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PubMed1997

Clinical testing of the statolith system in patients with Menière's disease.

The statolith ocular reflex induced by whole body roll was assessed in 10 patients with Meniere's disease who had undergone a standard vestibular test battery. Normal eye torsion was noted in 4 patients despite the presence of severe symptoms, and a caloric hyporeflexia in one patient. Eye torsion was diminished in one direction of body roll in 4 patients and in both directions of body roll in 2 patients. An abnormal asymmetry of eye torsion (> 16%) was found in the case of body roll towards the normal ear irrespective of whether a caloric asymmetry was found. An abnormal asymmetry of the statolith ocular reflex seems to indicate the side with the functional hearing loss. The results suggest that Meniere's disease might involve pathology of the statolith system. However, the absence of abnormal eye torsion does not imply normal vestibular function.

Adult↗
PubMed1999

Case report on SHORT syndrome.

The acronym SHORT was first used by Gorlin et al. (1975) and Sensenbrenner et al. (1975) to define a recognizable pattern of features, consisting of Short Stature, Hyperextensibility of joints and/or inguinal Hernia, Ocular depression, Rieger anomaly, and Teething delay. Other features characteristic of the syndrome included intrauterine growth retardation (IUGR), slow weight gain, frequent illness, triangular face, anteverted ears, telecanthus, deeply set eyes, wide nasal bridge, hypoplastic alae nasi, chin dimple, micrognathia, clinodactyly, partial lipodystrophy, hearing loss, functional heart murmur, delayed bone age, delayed speech, normal intellect, glucose intolerance, and insulinopenic diabetes. To our knowledge 19 cases of SHORT syndrome have been reported (Gorlin et al., 1975; Sensenbrenner et al., 1975; Aarskog et al., 1983; Toriello et al., 1985; Lipson et al., 1989; Schwingshandl et al., 1993; Verge et al., 1994; Bankier et al., 1985; Brodsky et al., 1996; Sorge et al., 1996; Haan and Morris, 1998). We report the twentieth patient diagnosed with SHORT syndrome who presented with growth retardation, sensorineural hearing loss, and minor dysmorphic features, consistent with the phenotype described for this syndrome.

Abnormalities, Multiple↗
PubMed1996

Transient-evoked otoacoustic emissions. Helpful tool in the detection of pseudohypacusis.

The object of the present study was to demonstrate the use of TEOAEs for diagnosing pseudohypacusis. Thirty-four patients were investigated for suspected pseudohypacusis based on the case history and clinical and audiological findings. TEOAEs confirmed the diagnosis in all cases except four patients with a pre-existing organic hearing loss exceeding 25-30 dB HL. All the remaining 30 patients showed normal TEOAE responses except four of the ears in which final assessment revealed hearing threshold lower than 30 dB in the middle frequency region. The method can be time-saving when performed at the first consultation. With its element of surprise, the patient can be confronted with the existence of a functional hearing loss. This frequently results in marked threshold improvement on repeat pure-tone audiometry.

Acoustic Stimulation↗
PubMed1989

Hearing loss in prison inmates.

The incidence of hearing disorders in 34 State Penitentiary prison inmates all with a previous history of drug abuse were investigated. Subjects were evaluated using routine pure-tone air conduction audiometry, immittance measures, and short-latency auditory brain stem responses. Of the 34 inmates, 20 (58.8%) demonstrated normal bilateral peripheral hearing sensitivity, whereas 10 (29.4%) inmates presented with some degree of hearing impairment. In addition, the conflicting results of elevated pure-tone thresholds with normal immittance measures and normal ABR findings suggested that four (11.8%) subjects exhibited functional hearing loss. The results of this study support the reported high incidence of hearing loss in the prison population. The synergistic effects of drug abuse, noise exposure, and head trauma as possible contributing factors are discussed.

Adolescent↗
PubMed1990

Hearing loss in the nursing home. An institutional issue.

One hundred ninety-eight nursing home admissions underwent audiometric assessment and answered questions regarding hearing difficulty in common listening situations. Twenty-four percent demonstrated normal thresholds (0-25 dB) in the speech frequencies with only mild losses at 4000 Hz. Fifty-four percent had normal thresholds through the speech frequencies with moderate to profound losses at higher frequencies, or mild losses (26-40 dB) in the speech frequencies. Such persons would be at risk in daily listening situations. Twenty-two percent had moderate or greater losses (greater than 40 dB) through the entire frequency range. Sixteen percent of this significantly impaired group were not identified as having a hearing loss by either the admitting RN or physician. Sixty percent of residents reported trouble in a group if they could not see the speaker's face, when watching television, and/or when using the telephone. The data indicate that a systematic hearing screening program is the most reliable means of identifying hearing loss and functional hearing handicap on an individual basis. The high prevalence of hearing problems suggests that this handicap also needs to be addressed from the perspective of institutional policy. Staff training and environmental modification should be undertaken to give hearing support to the entire nursing home population.

Aged↗
PubMedDate not supplied

The N1 response and its applications.

Some properties and applications of the N1-P2 complex (100-200 ms latency) are reviewed. N1-P2 is currently the auditory-evoked potential (AEP) of choice for estimating the pure-tone audiogram in certain subjects for whom a frequency-specific, non-behavioural measure is required. It is accurate in passively cooperative and alert older children and adults. Although generally underutilized, it is an excellent tool for assessment of functional hearing loss, and in medicolegal and industrial injury compensation claimants. Successful use of N1-P2 requires substantial tester training and skill, as well as carefully designed and efficient measurement protocols. N1-P2 reflects conscious detection of any discrete change in any subjective dimension of the auditory environment. In principle, it could be used to measure almost any threshold of discriminable change, such as in pitch, loudness, quality and source location. It is established as a physiologic correlate of phenomena such as the masking level difference. Thus, N1-P2 may have many applications as an 'objective' proxy for psychoacoustic measures that may be impractical in clinical subjects. Advances in dipole source localization and in auditory-evoked magnetic fields (AEMFs) have clarified the multiple, cortical origins of N1 and P2. These potentials are promising tools for the neurophysiologic characterization of many disorders of central auditory processing and of speech and language development. They also may be useful in direct 'functional imaging' of specific brain regions. A wide variety of potential research and clinical applications of N1 and P2, and considerable value as part of an integrated, goal-directed AEP/AEMF measurement scheme, have yet to be fully realized.

Adult↗
PubMed1994

[Vestibular function in bilateral idiopathic progressive sensorineural hearing loss].

To investigate vestibular function in patients with bilateral progressive sensorineural hearing loss (BPSHL), we examined 5 cases using electronystagmography. Cases 1, 2 and 3 were adult type, cases 4 and 5 were juvenile type. All patients had dizzy spells in the early stage of the disease, and showed spontaneous nystagmus. Bilateral reduction of caloric response and very low VOR (vestibulo-ocular reflex) gain on rotation testing were observed in cases 1, 2 and 3. Case 4 showed right canal paresis on the caloric test and left directional preponderance on the rotation test. Case 5 showed good responses to both tests. Optokinetic afternystagmus (OKAN) in cases 2, 3 and 5 was not brisk, and cases 1 and 4 showed directional preponderance of OKAN. Although OKAN is useful for detecting directional preponderance, it does not always reflect peripheral vestibular function. We suggest the existence of two types of BPSHL which correlate with vestibular function. One type is associated with high grade vestibular dysfunction while, in the other, vestibular function is fair.

Adolescent↗
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↗
PubMed1999

A stepwise approach to the diagnosis and treatment of hereditary hearing loss.

What To Do Do suspect a genetic cause in all cases of hearing loss. Do develop a working knowledge of common types of HHI that you may draw on to aid in diagnosis. Do think of HHI when the audiogram reveals a hearing loss with a "cookie bite" configuration. Do refer the infant to a geneticist in cases where you suspect a syndromic HHI, a nonsyndromic HHI, and in cases of "cryptogenic" hearing loss where an underlying HHI may be present. Often, the associated symptoms are subtle and best detected by a professional who deals with these issues on a daily basis. Do get the infant or family plugged into an audiologist or otolaryngologist and speech pathologist who will preferably work as a team to maximize aural rehabilitation and ensure serial follow-up. It is never too early to fit a child with hearing aids. Do refer to the HHIRR center at Boys Town. Do refer to the correct "deaf" organization or "blind-deaf" organization. Do think about working up other siblings or family members. Do keep in mind that some members of the "deaf society" may regard deafness as an alternative lifestyle and may not be amenable to their child's referral for additional workup and aural rehabilitation. What Not To Do Do not assume the child is deaf and nothing can be done. Do not wait until the child is older to refer to an otolaryngologist, speech therapist, and audiologist. Do not order a sonogram. Do not order a temporal bone CT scan on newborns. Do not forget about other siblings who may have a similar pathology. Do not forget that some forms of HHI can present beyond infancy. The pediatrician is the front line and can play a major role in the diagnosis, workup, and treatment of HHI. Armed with the proper degree of suspicion, careful elicitation of family history, meticulous physical examination, evaluation of the audiogram, and adequate fund of knowledge of common types of genetic deafness, the pediatrician can make a timely diagnosis and appropriate referrals. This avoids delay in detection of significant hearing impairment and the associated lack of essential skills in speech, language, and social interaction. No child is too young to have some type of hearing assessment. Early detection and intervention are best done with a multidisciplinary team approach with a neonatologist or pediatrician, audiologist, speech therapist, and otolaryngologist. In the future, blood tests using genetic probes may be available to screen for many types of HHI.

Child↗
PubMed1989

Practices and attitudes related to hearing: a survey of executives.

Executives were surveyed about their hearing health-care practices and attitudes toward hearing loss (N = 140, mean age = 49, number of males = 133). In regard to hearing health-care practices, about one-third of the executives had not had a hearing test during the past 5 years. Fifty-one percent of the executives reported that a hearing test was conducted or recommended during their annual physical examinations. Twenty (14%) of the executives rated their hearing as fair or poor, but only two of them wore hearing aids. Executives who reported poorer hearing were less likely to have had recent hearing tests. When seeking a hearing evaluation, the executives indicated a two-to-one preference for medical doctors over audiologists--none preferred hearing aid dispensers. Regarding attitudes, approximately 90% of the executives felt that hearing aids were effective, but only two-thirds disagreed with the stereotype that hearing aids connote old age. Executives who tended to equate hearing aids with old age were less likely to be aware of hearing-impaired employees in their companies. Implications of these findings are discussed.

Administrative Personnel↗
PubMed2007

Protection against cisplatin-induced ototoxicity by adeno-associated virus-mediated delivery of the X-linked inhibitor of apoptosis protein is not dependent on caspase inhibition.

HYPOTHESIS: Gene therapy with an adeno-associated viral (AAV) vector encoding the X-linked inhibitor of apoptosis protein (XIAP) in an animal model of cisplatin-induced ototoxicity can elucidate apoptotic pathways in the inner ear. BACKGROUND: Cisplatin is limited clinically by ototoxicity associated with apoptosis in the inner ear. The relevant intracellular apoptotic pathways, however, are unknown. XIAP is an antiapoptotic protein that both inhibits caspases and reciprocally regulates the proapoptotic Smac/Omi proteins. AAV-mediated delivery of various XIAP mutants could distinguish between these antiapoptotic pathways in the ear and further the development of specific reagents for gene therapy- mediated prevention of cisplatin-induced ototoxicity. METHODS: We administered unilaterally through the round-window AAV-harboring genes encoding wild-type dXIAP, yellow fluorescent protein, or either of two dXIAP point mutants-one deficient in caspase inhibition (dXIAP-d) and the other additionally deficient in the binding of Smac/Omi (dXIAP-t). All rats received a 3-day systemic course of cisplatin. Functional hearing loss was measured by shifts in auditory brainstem response (ABR) thresholds after cisplatin treatment, and hair-cell loss was assessed by whole-mount phalloidin staining of cochlear turns. RESULTS: Uninjected ears universally displayed high-frequency-specific hair-cell loss and ABR threshold shifts upon cisplatin treatment. Although yellow fluorescent protein had no effect, ears injected with dXIAP exhibited 68% less ABR threshold shift at 32 kHz and 50% less basal-turn outer-hair-cell loss compared with contralateral untreated ears. This protection was maintained in ears injected with dXIAP-d but was abolished in those expressing dXIAP-t, which is incapable of blocking Smac/Omi. CONCLUSION: Hair-cell apoptosis induced by cisplatin involves the Smac/Omi pathway. Thus, gene therapy with either wild-type dXIAP or Smac/Omi-selective dXIAP-d may be effective to protect against cisplatin-mediated ototoxicity.

Animals↗