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PubMed2000

Neurogenin 1 null mutant ears develop fewer, morphologically normal hair cells in smaller sensory epithelia devoid of innervation.

The proneuronal gene neurogenin 1 (ngn1) is essential for development of the inner-ear sensory neurons that are completely absent in ngn1 null mutants. Neither afferent, efferent, nor autonomic nerve fibers were detected in the ears of ngn1 null mutants. We suggest that efferent and autonomic fibers are lost secondarily to the absence of afferents. In this article we show that ngn1 null mutants develop smaller sensory epithelia with morphologically normal hair cells. In particular, the saccule is reduced dramatically and forms only a small recess with few hair cells along a duct connecting the utricle with the cochlea. Hair cells of newborn ngn1 null mutants show no structural abnormalities, suggesting that embryonic development of hair cells is independent of innervation. However, the less regular pattern of dispersal within sensory epithelia may be caused by some effects of afferents or to the stunted growth of the sensory epithelia. Tracing of facial and stato-acoustic nerves in control and ngn1 null mutants showed that only the distal, epibranchial, placode-derived sensory neurons of the geniculate ganglion exist in mutants. Tracing further showed that these geniculate ganglion neurons project exclusively to the solitary tract. In addition to the normal complement of facial branchial and visceral motoneurons, ngnl null mutants have some trigeminal motoneurons and contralateral inner-ear efferents projecting, at least temporarily, through the facial nerve. These data suggest that some neurons in the brainstem (e.g., inner-ear efferents, trigeminal motoneurons) require afferents to grow along and redirect to ectopic cranial nerve roots in the absence of their corresponding sensory roots.

Animals↗

PubMed1995

[Morphologic characteristic of substance P immunoreactivity in facial nerve of normal cats].

This paper presents the morphologic charactistic of substance P immunoreactivity (SP-IR) in the facial nerve and geniculate ganglion of normal cats by using immunohistochemical technique and immunoelectronic microscopy. SP-IR positive cells are more in geniculate ganglion than the other parts of the facial nerve. These cells are black. The sizes of their somata are different, and the shapes are round, elliptic or irregular. The somata are connected with each other by their central and peripheral synapses in the shapes of spider's web or root of a tree. Among the cells the bipolar and pseudounipolar cells are also found. SP-IR positive fibers curve and some of them have obvious expansive bodies, which are beaded, different in diameter. Under electronic microscope SP-IR positive cells show thicker electronic density than the others, and SP-IR positive products with thick electronic density are found in the nuclei, nucleomembranes, outer membranes of mitochndria and plasma. The nerve fibers are myelinated and the myelin sheath is concentric, and in the axoplasms there are nervous microfilament and a bit of mitochndria.

Animals↗

PubMed1998

Target pioneering and early morphology of the murine chorda tympani.

Many studies demonstrate that differentiation of certain sensory receptors during development is induced by their nerve supply. Thus the navigational accuracy of pioneering fibres to their targets is crucial to this process. The special gustatory elements of the facial and glossopharyngeal nerves are used extensively as model systems in this field. We examined the chorda tympani, the gustatory component of the facial nerve, to determine the precise time course of its development in mice. The transganglionic fluorescent tracer DiI was injected into the anterior aspect of the mandibular arch of fixed embryos aged between 30 and 50 somites (E10-E12). It was allowed to diffuse retrogradely via the geniculate ganglion to the brainstem for 4 wk, before the distribution of DiI was determined using confocal laser scanning microscopy. Geniculate ganglion cells were first labelled at the 34 somite stage (E10). Pioneering chorda tympani fibres that arise from these cells passed peripherally and followed an oblique course as they grew towards the mandibular arch. At the 36 somite stage (E10.5), the peripheral component followed an intricate postspiracular course and passed anteriorly to arch over the primitive tympanic cavity, en route to the lingual epithelium. From the 36 to 50 somite stages (E10.5-E12), it consistently traced in the fashion of a 'U' bend. The central fascicle also traced at the 36 somite stage (E10.5) and just made contact with the brainstem. At the 40 somite stage (E11), the central fibres clearly chose a route of descent into the spinal trigeminal tract and branched into the solitary tract. Pioneering chorda tympani fibres contact the lingual epithelium when the target is primordial. The lingual epithelium may be a source of a neurotropic factor that attracts peripheral chorda tympani fibres to the sites of putative papillae. However, the chorda tympani is probably not a vital influence on the subsequent differentiation of gustatory papillae, since the papillae are elaborated 5 d later at E15 in murine embryos. The early morphology of the nerve is true to the amniote vertebrate phenotype.

Animals↗

PubMed2003

Microsurgical anatomy of the perigeniculate ganglion area as seen from the middle cranial fossa approach.

The middle cranial fossa approach is useful for decompressing the perigeniculate ganglion area of the facial nerve in patients with serviceable hearing. The present study was designed to investigate the microsurgical anatomy of the perigeniculate ganglion area of the facial nerve from the point of view of the middle cranial fossa. We dissected 20 human temporal bones under a microscope using a middle fossa approach, and measured the angle between the lines drawn from the malleus head to the vertical crest and from the malleus head to the geniculate ganglion, and the distance from the malleus head to the geniculate ganglion. These were found to be 22.7 degrees +/- 2.2 degrees and 6.5 +/- 0.3 mm, respectively. Detailed knowledge about the microsurgical anatomy of the perigeniculate ganglion area of the facial nerve from the point of view of the middle cranial fossa is imperative for facial nerve decompression by a middle cranial fossa approach.

Cranial Fossa, Middle↗

PubMed2002

Geniculate neuralgia: long-term results of surgical treatment.

A rare cause of otalgia is geniculate neuralgia. In its most typical form, it is characterized by severe paroxysmal neuralgic pain centered directly in the ear. The pain can be of a gradual onset and of a dull, persistent nature, but occasionally it is sharp and stabbing. When the pain becomes intractable, an operation to surgically excise the nervus intermedius and geniculate ganglion via the middle cranial fossa approach is indicated. The purpose of this article is to review the long-term outcomes in 64 patients who were treated in this manner. Findings indicate that excision of the nervus intermedius and geniculate ganglion can be routinely performed without causing facial paralysis and that it is an effective definitive treatment for intractable geniculate neuralgia.

Earache↗

PubMed1998

Quantitative relationships between taste bud development and gustatory ganglion cells.

To determine whether patterns of taste bud innervation change during postnatal rat development, the number of geniculate ganglion cells that innervate single taste buds were quantified in adult and developing rats. While there was a large variation in numbers of ganglion cells that innervate individual taste buds, there was a high degree of organization in the system. Namely, the number of labeled geniculate ganglion cells innervating a taste bud was highly correlated with the size of the taste bud. This relationship between taste bud size and number of innervating ganglion cells develops over a prolonged postnatal period and is not established until postnatal day 40 (P40), when taste buds reach their adult size. In a second series of experiments, we sought to determine whether neural rearrangement of chorda tympani neurons is responsible for the development of this relationship by double-labeling single taste buds at different ages. We found that the number of ganglion cells innervating individual taste buds on P10 predicts the size that taste buds become by P40. This finding suggests that neural rearrangement is not responsible for establishing the relationship between taste bud size and the number of innervating ganglion cells during development. More importantly, it strongly suggests that the 'neural template' for the mature innervation pattern is determined during early postnatal development.

Animals↗

PubMed1979

Total facial nerve exploration: transmastoid, extralabyrinthine, and subtemporal indications and results.

Increasingly, surgeons are using a middle fossa approach though craniotomy to reach the labyrinthine segment of the facial nerve and geniculate ganglion in patients with intact hearing. This paper describes a transmastoid operation that provide exposure of the labyrinthine segment of the facial nerve without performance of a craniotomy. In this procedure the geniculate ganglion and labyrinthine segments of the facial nerve were exposed, while cochleovestibular function was spared; recovery of the facial nerve in patients with Bell's palsy or herpes zoster oticus (even the patients with a dry eye) was favourably influenced.

Facial Nerve↗

PubMed2000

Neuron/target matching between chorda tympani neurons and taste buds during postnatal rat development.

During postnatal development, a relationship is established between the size of individual taste buds and number of innervating neurons. To determine whether rearrangement of neurons that innervate taste buds establishes this relationship, we labeled single taste buds at postnatal day 10 (P10) and again at either P15, P20, or P40 with retrograde fluorescent neuronal tracers. The number of single- and double-labeled geniculate ganglion cells was counted, and the respective taste bud volumes were measured for the three groups of rats. The current study replicates findings from an earlier report demonstrating that the larger the taste bud, the more geniculate ganglion cells that innervate it. This relationship between taste bud size and number of innervating neurons is not apparent until P40, when taste bud size reaches maturity. These findings are extended here by demonstrating that the number of neurons that innervate taste buds at P10, when taste bud size is small and relatively homogeneous, predicts the size that the respective taste bud will become at maturity. Moreover, while there is some neural rearrangement of taste bud innervation from P10 to P40, rearrangement does not impact the relationship between taste bud size and innervating neurons. That is, the neurons that maintain contact with taste buds from P10 through P40 accurately predict the mature taste bud size. Therefore, the size of the mature taste bud is determined by P10 and relates to the number of sensory neurons that innervate it at that age and the number of neurons that maintain contact with it throughout the first 40 days of postnatal development.

Age Factors↗

PubMed2001

Neural hearing loss in a child with poliomyelitis: a histopathological study.

The temporal bones of a 26-month-old white female with a paralytic syndrome clinically and pathologically identical to poliomyelitis were examined. The aetiological agent was unknown although a non-poliomyelitis enterovirus infection seemed likely. There was a complete absence of the cochlear neurons and substantially reduced peripheral and central axons with loss of some inner hair cells but preservation of outer hair cells. Scarpa's ganglion, and the geniculate ganglion were partially atrophied. The saccule and utricle were mildly dilated and Reissner's membrane of the apical turn was bulging. In two previous audiological studies a 10--20 dB bilateral sensorineural hearing loss was found in poliomyelitis patients and a neuronal lesion was postulated which is now supported by our findings. This is a rare example of an almost pure neural hearing loss.

Child, Preschool↗

PubMed1991

Inflammatory pseudotumor of the facial nerve as a cause of recurrent facial palsy: case report.

This is a report of a case presenting recurrent facial palsy due to inflammatory pseudotumor of the facial nerve. The patient was a 41-year-old man suffering from facial palsy on the right side once in 1983, which recurred in 1988. The initial facial palsy was cured after conservative treatments including steroid administration. Three months after the recurrence, the same treatments were attempted without noticeable effect. He was referred to our clinic two and half months later. Electromyographic examination indicated pronounced denervation of the facial nerve. Imaging study including high resolution CT and MRI revealed a mass lesion around the geniculate ganglion. Facial neuroma was initially suspected. Surgical exploration revealed a tumor involving the geniculate ganglion and the horizontal portion of the facial nerve. The tumor was resected through a combined transmastoid and middle cranial fossa approach, followed by nerve grafting. Histologically, the tumor proved to be an inflammatory pseudotumor originating from the nerve sheath, although the etiologic factor causing the inflammation was not verified.

Adult↗

PubMed2002

Swelling of the intratemporal facial nerve in Ramsay Hunt syndrome.

Although Ramsay Hunt syndrome is one of the most important diseases causing peripheral facial palsy, the detailed pathology of the disease in the intratemporal facial nerve remains unclear. The purpose of this study was to increase knowledge of the pathogenesis of the syndrome by means of surgical findings. Between April 1976 and March 1997 we performed subtotal decompression of the facial nerve in 74 patients with severe Ramsay Hunt syndrome. The grade of nerve swelling was assessed using a microscope and recorded in a standardized form. The relationships between nerve swelling, the timing of surgery and the swelling of each segment were analyzed. Pronounced neural swelling, involving the geniculate ganglion and the horizontal segment, was consistent finding in the acute phase. Although the incidence of pronounced swelling of the horizontal segment gradually declined with time after onset, in most cases nerve swelling persisted even beyond the 16th week after onset. These data suggest that diffuse viral neuritis occurs throughout the intratemporal facial nerve. We assume that the viral inflammatory swelling involving the geniculate ganglion and horizontal segment is mostly responsible for the acute facial palsy in the acute phase.

Adult↗

PubMed1992

Normal and abnormal pathfinding of facial nerve fibers in the chick embryo.

Development of the facial nerve was studied in normal chicken embryos and after surgical disruption of ingrowing sensory facial nerve fibers at 38-72 h of incubation. Disruption of facial nerve fibers by otocyst removal often induced a rostral deviation of the facial nerve and ganglion to the level of the trigeminal ganglion. Cell bodies of the geniculate ganglion trailed their deviating neurites and occupied an abnormal rostral position adjacent to the trigeminal ganglion. Deviating facial nerve fibers were labeled with the carbocyanine fluorescent tracer DiI in fixed tissue. Labeled fibers penetrated the cranium adjacent to the trigeminal ganglion, but they did not follow the trigeminal nerve fibers into the brain stem. Rather, after entering the cranium, they projected caudally to their usual site of entrance and proceeded towards their normal targets. This rostral deviation of the facial nerve was observed only after surgery at 48-72 h of incubation, but not in cases with early otocyst removal (38-48 h). A rostral deviation of the facial nerve was seen in cases with partial otocyst removal when the vestibular nerve was absent. The facial nerve followed its normal course when the vestibular nerve persisted. We conclude that disruption of the developing facial pathway altered the routes of navigating axons, but did not prevent pathfinding and innervation of the normal targets. Pathfinding abilities may not be restricted to pioneering axons of the facial nerve; later-developing facial nerve fibers also appeared to have positional information. Our findings are consistent with the hypothesis that navigating axons may respond to multiple guidance cues during development. These cues appear to differ as a function of position of the navigating axon.

Acetylcholinesterase↗

PubMed2005

In vivo recordings from rat geniculate ganglia: taste response properties of individual greater superficial petrosal and chorda tympani neurones.

Coding of gustatory information is complex and unique among sensory systems; information is received by multiple receptor populations located throughout the oral cavity and carried to a single central relay by four separate nerves. The geniculate ganglion is the location of the somata of two of these nerves, the greater superficial petrosal (GSP) and the chorda tympani (CT). The GSP innervates taste buds on the palate and the CT innervates taste buds on the anterior tongue. To obtain requisite taste response profiles of GSP neurones, we recorded neurophysiological responses to taste stimuli of individual geniculate ganglion neurones in vivo in the rat and compared them to those from the CT. GSP neurones had a distinct pattern of responding compared to CT neurones. For example, a small subset of GSP neurones had high response frequencies to sucrose stimulation, whereas no CT neurones had high response frequencies to sucrose. In contrast, NaCl elicited high response frequencies in a small subset of CT neurones and elicited moderate response frequencies in a relatively large proportion of GSP neurones. The robust whole-nerve response to sucrose in the GSP may be attributable to relatively few, narrowly tuned neurones, whereas the response to NaCl in the GSP may relate to proportionately more, widely tuned neurones. These results demonstrate the diversity in the initial stages of sensory coding for two separate gustatory nerves involved in the ingestion or rejection of taste solutions, and may have implications for central coding of gustatory quality and concentration as well as coding of information used in controlling energy, fluid and electrolyte homeostasis.

Action Potentials↗

PubMed1999

Petrosal cholesteatoma: management considerations for minimizing morbidity.

OBJECTIVE: This study aimed to analyze the surgical treatment of patients presenting with petrosal cholesteatoma. STUDY DESIGN: The study design was a retrospective case review. SETTING: The study was conducted at a tertiary referral center. PATIENTS: Patients were referred to the University Department of Otolaryngology, Manchester Royal Infirmary, with surgically confirmed petrosal cholesteatoma. INTERVENTION: Interventions were diagnostic and therapeutic. MAIN OUTCOME MEASURES: Postoperative facial function, hearing loss, recurrent cholesteatoma, and petrous cavity morbidity in relation to preoperative signs, intraoperative findings, and surgical techniques were measured. RESULTS: Twenty-five patients were treated between 1979 and 1997. Complete preoperative facial paralysis was always associated with bony erosion around the geniculate ganglion, and facial nerve ischemia was thought to be an important factor. Resection of the ischemic nerve segment and end-to-end anastomosis over the posterior fossa dura allowed full removal of cholesteatoma matrix and reinnervation along a healthy and vascularized nerve. In five of the seven cases with recurrent disease, cholesteatoma was localized to the intrapetrous carotid or geniculate ganglion. The introduction of endoscopic techniques has allowed assessment of the full extent of disease and removal of cholesteatoma matrix missed using conventional microscopic visualization. Bipolar diathermy to areas of cholesteatoma, densely adherent to dura, is effective in destroying squamous epithelium. Patients with cavity obliteration and blind sac closure had minimal postoperative morbidity, and current magnetic resonance imaging techniques are effective in diagnosing and delineating recurrence. CONCLUSIONS: This study illustrates a gradual change in the treatment of patients with petrosal cholesteatoma. By defining the main areas of patient morbidity and using both recent technical advances and alternative surgical techniques, further improvement in patient outcome is envisaged.

Adolescent↗

PubMed1997

Molecular temporal bone pathology: II. Ramsay Hunt syndrome (herpes zoster oticus).

In 1907 J. Ramsay Hunt suggested that herpes zoster oticus resulted from a geniculate ganglionitis; however, many contemporary authors believe that this disorder represents a neuritis or polycranial neuropathy. Herpes varicella-zoster viral (VZV) DNA was identified, using the polymerase chain reaction, in archival celloidin-embedded temporal bone sections from two patients who clinically had Ramsay Hunt syndrome (herpes zoster oticus). The presence of VZV was confirmed by sequencing the PCR products. These experiments demonstrated that VZV genomic DNA was present in the geniculate ganglion of the side with facial paralysis and cutaneous recrudescence in both patients and in the clinically unaffected side in patient 1. In addition, patient 2 had a sudden hearing loss and was found to have VZV genomic DNA in sections from the affected side containing the spiral ganglion, Scarpa's ganglion, organ of Corti, and macula of the saccule. No VZV genomic DNA was identified in temporal bone sections from five patients with Bell's palsy and ten patients without evidence of otologic disease. In this study, the histopathology of these two cases yielded complementary information regarding the role of VZV in herpes zoster oticus. These data suggest that in patients with Ramsay Hunt syndrome, latent VZV is located in the geniculate ganglia and may be present in the auditory and vestibular primary afferent ganglia in some patients.

DNA, Viral↗

PubMed1981

Surgery of the facial nerve.

The diagnosis and management of facial nerve disorders are challenging aspects of neurotological practice. In Bell's palsy and Herpes Zoster oticus facial nerve decompression is indicated in approximately 10 per cent of cases within the first three weeks if 90-95 per cent degeneration has occurred. The presence of a dry eye determines whether the lesion is proximal to the geniculate ganglion and a middle fossa approach is necessary. Injury to the facial nerve occurs most commonly as a result of temporal bone fractures, from either blunt trauma or penetrating wounds. Occasionally iatrogenic injury occurs during ear surgery. Injury resulting in immediate and total facial paralysis with degeneration confirmed by appropriate electrical testing requires facial nerve exploration. In the presence of an injury proximal to the geniculate ganglion and hearing preservation, exploration of the nerve via middle fossa approach is indicated. If the hearing is lost then the translabyrinthine approach is appropriate. In the event the entire interosseous segment requires exposure, both mastoid and middle fossa exposures are required. If the traumatized facial nerve has not been divided it is decompressed proximal and distal to the injury site. In the event the nerve is divided, a direct anastomosis is the ideal treatment. Rerouting is necessary to achieve adequate length for the anastomosis. Nerve grafting is done when required.

Accidents, Traffic↗

PubMed2000

Involvement of nitric oxide synthase in the physiology and pathophysiology of facial nerve function and dysfunction.

To date few reports have discussed the presence and function of nitric oxide (NO) in structures of the facial nerve. We performed nicotinamide adenine dinucleotide phosphate (NADPH-d)-diaphorase-histochemistry and immunohistochemistry on the intratemporal portion of the facial nerve, including the geniculate ganglion, of guinea pigs using specific antibodies to the three known isoforms of NO synthase and soluble guanylyl-cyclase (sGC). Normal facial nerves were compared to those treated intratympanically with bacterial lipopolysaccharides (LPS) and tumor necrosis factor-alpha (TNF-alpha). Both constitutive NOS isoforms and sGC could be detected in the bipolar ganglion cells of normal animals, while the inducible isoform (iNOS or NOS II) was not found. Endothelial NOS (NOS III) and sGC were present in blood vessels and were predominantly found in the perineurial sheath and less in the endoneurium. sGC could be detected in all fibers in a cross section of the facial nerve. LPS and TNF treatment led to the detection of iNOS in the perikaryia of the geniculate ganglion and the perineural sheath. These findings imply that NO may be involved in neurotransmission at least in the visceroafferent system. NO regulates vascular tone of nutrient blood vessels in the perineural sheath and endoneurium. The presence of sGC indicates that NO acts via its second messenger cGMP. NOS II expression may be a contributing factor to facial nerve palsy via two different mechanisms: NOS II-generated NO may lead to an overstimulation of the visceroefferent nerve fibers and motor fibers of the facial nerve. Dysregulation in facial nerve blood vessels could lead to edema and elevated pressure on the nerve within its osseous canal.

Animals↗

PubMed1991

Geniculate neuralgia: the surgical management of primary otalgia.

Intractable, unexplained deep-ear pain presents a rare, albeit significant problem in otolaryngological and neurosurgical practice. The authors review their experience with 18 cases of primary otalgia during the past 15 years. A total of 31 surgical procedures were performed. Seventeen patients had sequential rhizotomies and one patient had microvascular decompression alone. Based on the clinical diagnosis, the nerves sectioned were singly or in combination: the nervus intermedius (14 patients), geniculate ganglion (10 patients), ninth nerve (14 patients), 10th nerve (11 patients), tympanic nerve (four patients), and chorda tympani nerve (one patient). Microvascular decompression of the involved nerves was undertaken in nine patients, in whom vascular loops were discovered. Adhesions (six patients), thickened arachnoid (three patients), and benign osteoma (one patient) were other intraoperative abnormalities noted. The overall success of these procedures in providing pain relief was 72.2%, and the mean follow-up period was 3.3 years (range 1 month to 14.5 years). There was no surgical mortality. Expected side effects were: decreased lacrimation, salivation, and taste related to nervus intermedius nerve section, and transient hoarseness and diminished gag related to ninth and 10th nerve section. Four patients developed sequelae consisting of sensorineural hearing loss, vertigo, and transient facial nerve paresis. One patient had a cerebrospinal fluid leak and another developed aseptic meningitis as postoperative complications. Except when primary glossopharyngeal neuralgia is the working diagnosis, a combined posterior cranial fossa-middle cranial fossa approach is recommended for adequate exploration and/or section of the fifth, ninth, and 10th cranial nerves as well as the geniculate ganglion and nervus intermedius.

Adult↗