Noncongenital hereditary hearing loss in children. Prospective documentation

Younger siblings of children with sensorineural hearing loss of possible hereditary cause underwent interval audiologic examination. Seven siblings (in unrelated families) were found to have progressive sensorineural hearing loss despite early audiograms documenting normal hearing levels for age. Continued testing of these children allowed for early identification and intervention. We advocate regular otolaryngologic and audiologic follow-up even after normal audiologic assessments are made for younger siblings of children with documented sensorineural hearing loss, unless a definite nongenetic origin of the hearing loss in the older child is known. Recessive sensorineural hearing loss with onset in infancy or childhood may present with no antecedent family history and with normal behavioral audiograms early in life.     

Sensorineural hearing loss and Kawasaki disease: a prospective study

PURPOSE: Kawasaki disease (KD) is an acute, self-limited vasculitis of infants and children that is now the most common cause of acquired heart disease in the pediatric age group in the United States and Japan. Reports have documented the association of acute KD with sensorineural hearing loss. To assess the prevalence of hearing loss following acute KD in a geographically and ethnically diverse population, a prospective, multicenter study of hearing loss in patients with KD was conducted. MATERIALS AND METHODS: Patients with acute KD were enrolled in 7 clinical centers and underwent a primary audiologic evaluation within 30 days of the onset of fever. Patients were subsequently reevaluated after resolution of the acute phase of the disease. A questionnaire assessing risk factors for hearing loss was also administered. RESULTS: A total of 62 patients were evaluated during the 29-month study period. At the first audiologic evaluation, 19 patients (30.6%) had sensorineural hearing loss, 6 patients (9.7%) had conductive hearing loss, 17 patients (27.4%) had normal hearing, and 20 patients (32.3%) had inconclusive studies. Overall, 2 of 36 patients (5.5%) had sensorineural hearing loss documented on their second audiologic evaluation. No risk factors for hearing loss were identified by the questionnaire. CONCLUSIONS: Transient sensorineural hearing loss (20 to 35 dB) is a frequent complication of acute KD and may be related to salicylate toxicity in some patients. Persistent sensorineural hearing loss is uncommon. Parents and primary care providers should be made aware of the potential for persistent sensorineural hearing loss following resolution of KD, but routine audiologic screening of this patient population does not appear to be warranted.     

Congenital perilymphatic fistula: an overlooked diagnosis?

Congenital perilymphatic fistula (CPLF) often is the sole cause of sensorineural hearing loss in children that is both progressive and subtle and, therefore, can elude detection by physicians. A 3-year prospective study of 244 children with sensorineural hearing loss of unknown etiology demonstrated that at least 6% (15/244) had CPLF. Close audiologic monitoring and radiologic imaging of the temporal bone of children with sensorineural hearing loss are recommended to detect CPLF.     

Audiologic and otologic phenotype in children with Duane's Retraction Syndrome: A rare ophthalmologic disorder

ObjectiveThe focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with Duane's Retraction Syndrome and to relate these measures to patient demographics and other otologic and audiologic factors.Study designRetrospective AudGen Database analysisSettingTertiary academic referral centerPatientsPediatric patients in AudGenDB with a diagnosis of Duane's Retraction Syndrome (DRS).InterventionsAppropriate audiologic, otologic, and demographic data were recorded.Main outcome measureSeventy-nine patients (n = 79) met inclusion criteria. The first encounter with available audiometric data or the first encounter with hearing loss were documented. Audiograms were stratified by type and severity of hearing loss, and common associated medical issues were documented.Results57 children had normal hearing; 22 had hearing loss. 9 ears had pure conductive hearing loss, 1 had pure sensorineural, 14 ears had components of both, and 79 had hearing loss that could not be specified. Multivariate regression revealed episodes of chronic otitis and craniofacial anomalies are associated with worse hearing loss.ConclusionThis study presents a detailed characterization of hearing loss in patients with Duane's retraction syndrome. Conductive and sensorineural hearing loss are both prevalent among these children. Careful and early audiologic evaluation of all patients with DRS is important.     

Prognostic factors of profound idiopathic sudden sensorineural hearing loss

Profound idiopathic sudden sensorineural hearing loss is thought to have a poor prognosis, but few studies have focused on this condition. We aimed to assess the impact of patient factors, audiologic parameters, and salvage intratympanic steroid injection therapy on the prognosis of profound idiopathic sudden sensorineural hearing loss. The demographic, clinical, and audiologic data, degree of hearing recovery, and efficacy of intratympanic steroid injection therapy in 576 patients with profound idiopathic sudden sensorineural hearing loss (mean age 56.2 ± 14.9 years) who had been admitted at four tertiary referral centers between 2000 and 2011 were retrospectively reviewed. The mean hearing level at the initial presentation was 108.1 ± 9.5 dB. Many patients experienced vertigo (52.1 %) and tinnitus (77.4 %). At the 2-month follow-up, 172 (29.8 %) patients showed some degree of hearing recovery, but only 21 (3.6 %) patients recovered normal hearing. Further, the 116 patients who had received salvage intratympanic steroid injections showed a better audiologic outcome (improvement, 26.1 ± 24.3 vs. 15.7 ± 22.1 dB; P = 0.000) than those who had not (n = 429). In conclusion, a higher degree of hearing loss at the initial presentation indicates a poorer prognosis. Salvage intratympanic steroid injection therapy may improve the hearing of patients with profound idiopathic sudden sensorineural hearing loss after the failure of systemic steroid therapy.     

Sudden sensorineural hearing loss following manipulation of the cervical spine

Review of the otolaryngologic literature reveals no reports of sudden sensorineural hearing loss resulting from manipulation of the cervical spine. Indeed, in previously reported cases of vertebrobasilar artery injury following spinal manipulation, hearing loss has received little attention. Two patients with sudden sensorineural hearing loss following cervical spine manipulation are presented. The audiologic findings and cerebral arteriograms are reviewed and treatment is discussed. A review of the anatomy and pathophysiology is also included, and a possible mechanism of injury to the vertebral artery is proposed.     

Incidence of coexisting sensorineural hearing loss and secretory otitis media

There are currently no standard guidelines for assessing hearing in children who are evaluated for tympanostomy tubes. We describe the results of audiologic testing on 587 children, age 2 months to 17 years admitted to Pediatric Otolaryngology Department Bia?ystok for treatment of secretory otitis media. Ten children (1.7%) were found to have previously unrecognized sensorineural hearing loss. In four cases total unilateral deafness, in six others moderate to severe sensorineural bilateral hearing loss was diagnosed. Three other children referred to our clinic as sensorineural hearing loss were found to have secretory otitis media as the only or coexisting cause of deafness. Results of our study show the importance of age--appropriate hearing assessment as part of diagnostic procedure for secretory otitis media.     

Mass hearing screening in kindergarten students

OBJECTIVE: To determine the types of hearing losses identified by mass hearing screenings in the public school system. DESIGN: Prospective observational. PARTICIPANTS: Students enrolled in kindergarten in the public school system. SETTING: Major metropolitan area. MAIN OUTCOME MEASURES: (1) hearing screening tests performed by speech pathologists using an audiometer in a quiet room; (2) formal audiologic testing including pure tone audiometry, tympanograms, speech discrimination, and acoustic impedance testing in a sound proof booth. RESULTS: 140 students failed hearing screening on two separate occasions; 91(65%) underwent formal audiologic testing at Milwaukee Public School, 43% demonstrated conductive hearing losses, 14% demonstrated sensorineural losses and 43% demonstrated normal hearing. CONCLUSION: Mass hearing screening in the school system (1) is useful for detecting transient conductive hearing losses, (2) detects a significant number of sensorineural hearing losses and (3) has a very poor follow-through by the families of those students identified with hearing loss through the screening program.     

Sudden sensorineural hearing loss: a review of diagnosis, treatment, and prognosis

Sudden sensorineural hearing loss (SSNHL) is commonly encountered in audiologic and otolaryngologic practice. SSNHL is most commonly defined as sensorineural hearing loss of 30 dB or greater over at least three contiguous audiometric frequencies occurring within a 72-hr period. Although the differential for SSNHL is vast, for the majority of patients an etiologic factor is not identified. Treatment for SSNHL of known etiology is directed toward that agent, with poor hearing outcomes characteristic for discoverable etiologies that cause inner ear hair cell loss. Steroid therapy is the current mainstay of treatment of idiopathic SSNHL in the United States. The prognosis for hearing recovery for idiopathic SSNHL is dependent on a number of factors including the severity of hearing loss, age, presence of vertigo, and shape of the audiogram.     

Use of the argon laser in the treatment of malleus fixation

This case demonstrates the use of the argon laser for ossicular mobilization. A preoperative audiologic evaluation revealed a severe conductive hearing loss, with a maximum air-bone gap. Since normal drilling procedures would result in a sensorineural hearing loss, the argon laser was chosen to remove a bony spur connecting the malleus to the posterior canal wall. When using the argon laser, no disarticulation of the incus and stapes is required. Postoperative audiologic evaluation revealed normal hearing sensitivity bilaterally.     

Auditory neuropathy in childhood

Objectives: Auditory neuropathy is a recently described disorder in which patients demonstrate hearing loss for pure tones, impaired word discrimination out of proportion to pure tone loss, absent or abnormal auditory brainstem responses, and normal outer hair cell function as measured by otoacoustic emissions and cochlear microphonics. We have identified eight pediatric patients having hearing deficits that are most likely due to a neuropathy of the eighth nerve. In this study, the results of audiologic testing performed with these eight children are described. Study Design: Retrospective review of audiologic findings in eight children with auditory neuropathy. Methods. Each subject was tested with pure tone and speech audiologic testing, auditory brainstem response, and click-evoked otoacoustic emissions. Results of these tests were tabulated and summarized. Results: Pure tone audiologic testing revealed five children with upsloping sensorineural hearing loss, two with high frequency loss, and one with a mild, flat configuration. Six children demonstrated poor word discrimination scores, and the other two had fair to good word discrimination. All eight subjects had normal distortion product and transient otoacoustic emissions. All eight children demonstrated absent or marked abnormalities of brainstem auditory evoked potentials. These findings suggest that while cochlear outer hair cell function is normal, the lesion is located at the eighth nerve. Conclusions: Recent advances in otoacoustic emissions testing permit differentiation of neural deafness from sensory deafness. This paper describes the clinical presentation and audiologic findings in pediatric auditory neuropathy, as well as the recommended management of these patients. Otolaryngologists should be aware of this disorder and implications for its management, which differs from treatment of sensorineural hearing loss. Key Words: Auditory neuropathy, childhood, hearing loss, auditory brainstem response, evoked otoacoustic emissions.     

Delayed endolymphatic hydrops: a case study

Delayed endolymphatic hydrops (DEH) is an unusual variation of Ménière's disease characterized by episodic vertigo that develops some time after the onset of a profound, typically unilateral sensorineural hearing loss. This case study describes a 48-year-old male who presented with complaints of episodic vertigo and disequilibrium 15 years following the onset of unilateral sensorineural hearing loss. The patient's history, audiologic findings, and vestibular evaluation led to the diagnosis of DEH. The case highlights the diagnostic and treatment challenges associated with this condition and focuses attention on principles that guide the audiologist in collecting evidence that aids in solving these challenges.     

热休克蛋白70抗体检测对自身免疫性聋诊断的意义

目的 分析自身免疫性聋的听力学特点，探讨用免疫印迹法(Western blot)检测热休克蛋白70抗体在自身免疫性耳聋诊断中的意义。方法自身免疫性聋14例28耳，其它感音神经性聋8例16耳，正常对照6例12耳，采用免疫印迹法检测各组受试者血清中的热休克蛋白70抗体，并分别作临床听力学检查。结果自身免疫性耳聋者纯音听力图多数为平坦型，TEOAE和／或DPOAE未引出，听性脑干反应多数正常，轻、中度耳聋者可引出镫骨肌声发射，该组8例(57．1％)热休克蛋白70抗体阳性。其它感音神经性聋者纯音听力图多数为高频减退型和平坦型，TEOAE和DPOAE未引出，重度耳聋者ABR未引出，轻、中度耳聋者可引出镫骨肌声发射，1例(12．5%)热休克蛋白70抗体阳性。正常对照组1例(16．7％)热休克蛋白70抗体阳性。结论免疫印迹法检测热休克蛋白70抗体对自身免疫性耳聋诊断有参考价值，对该病的确诊还应结合临床听力学特点。     

Auditory function in patients with surgically treated superior semicircular canal dehiscence.

OBJECTIVE: To characterize preoperative and postoperative audiologic findings in patients with superior semicircular canal dehiscence syndrome. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Patients with documented superior semicircular canal dehiscence syndrome (according to history, vestibular testing, and high-resolution computed tomography imaging) who underwent surgical repair of their dehiscence. INTERVENTION: Middle fossa craniotomy for superior semicircular canal plugging and/or resurfacing. MAIN OUTCOME MEASURES: Audiologic testing both before and after surgery with pure-tone threshold measurements of air and bone conduction. RESULTS: Twenty-nine subjects underwent surgical repair of superior semicircular canal dehiscence. Overall, there were no statistically significant differences by paired t test in hearing before or after surgery, in either air-conduction or bone-conduction thresholds, for 19 patients that had no previous surgical history. At least partial closure of air-bone gap was achieved in five patients. One patient with previous stapes surgery had significantly worse hearing both before and after canal repair compared with those without previous surgery. Two patients who had undergone previous middle fossa surgery with incomplete resolution of symptoms developed sensorineural hearing loss after revision surgery. Previous middle-ear exploration and tympanostomy tube placement did not seem to affect audiologic outcomes. Surgical hearing results did not differ according to method of canal repair (plugging versus resurfacing). CONCLUSION: Primary middle fossa repair of superior semicircular canal dehiscence is not associated with sensorineural hearing loss and, in some cases, can lead to normalization of conductive hearing loss. Revision middle fossa repair or previous stapes surgery may be associated with postoperative sensorineural hearing loss.     

Sensorineural hearing loss in Nigerian children with sickle cell disease

AIM: To study the prevalence and pattern of sensorineural hearing loss (SNHL) in Nigerian children with sickle cell disease (SCD). MATERIALS: Fifty-two children with SCD were assessed. Otologic examination and audiometric tests were performed. There were 36 males and 16 females. Their age ranged from 6 to 19 years. RESULT: Seven children had hearing impairment. After hemoglobin categorization, all the SCD patients were found to belong to the homozygous (HbSS) group. There were no HbSC nor other variants detected. Prevalence of SNHL was 13.4% in these patients but the general population prevalence rate found in a control group was 6.2%. CONCLUSION: SCD has many organ manifestations and complication among which is sensorineural hearing loss. Regular audiologic assessment, counselling and rehabilitation of these patients with hearing aids are recommended.     

Hearing loss among patients with Turner's syndrome: literature review

IntroductionTurner's syndrome (TS) is caused by a partial or total deletion of an X chromosome, occurring in 1:2,000 to 1:5,000 live born females. Hearing loss is one of its major clinical manifestations. However, there are few studies investigating this problem.ObjectivesTo review the current knowledge regarding the epidemiology, etiology, clinical manifestations and diagnosis of hearing impairment in patients with TS.MethodsA bibliographic search was performed in the Medline and Lilacs databanks (1980-2012) to identify the main papers associating Turner's syndrome, hearing impairment and its clinical outcomes.ConclusionsRecurrent otitis media, dysfunction of the Eustachian tube, conductive hearing loss during infancy and sensorineural hearing loss in adolescence are the audiologic disorders more common in ST. The karyotype appears to be important in the hearing loss, with studies demonstrating an increased prevalence in patients with monosomy 45,X or isochromosome 46,i(Xq). Morphologic studies of the cochlea are necessary to help out in the clarifying the etiology of the sensorineural hearing loss.     

Electrocochleographic findings in cases of autoimmune disease with sensorineural deafness

Objectives: To present electrocochleographic findings in patients with autoimmune disease (AD) with sensorineural deafness (ADSD), and to discuss the etiologies of sensorineural hearing loss (SNHL) in cases of ADSD. Methods: Study design is a retrospective review of electrocochleographic results of 26 patients with ADSD. To evaluate the electrocochleographic results, average SP/AP ratios were compared between ADSD and normal subjects. In the ADSD group, audiologic pattern, fluctuations in hearing and results of the glycerol test were also reviewed. Electrocochleography (ECoG) was recorded using the extratympanic method, and the SP to AP ratio (SP/AP ratio) was analyzed. Results: The mean of average SP/AP ratios in the ADSD groups (0.46) was significantly higher than that in normal subjects (0.27). Further, 17 of 29 affected ears in patients with ADSD showed fluctuating hearing loss. Eighteen ears showed low tone loss (rising and peak audiologic patterns). Only 5 of 26 ears (19.2%) showed positive results on glycerol test. There was no correlation between glycerol test results and hearing fluctuation or between glycerol test results and the SP/AP ratio on χ²-test. There was no tendency between audiologic pattern and glycerol test results or between audiologic pattern and the SP/AP ratio. Conclusion: These results suggest the etiologies of SNHL in cases of ADSD remain unclear. However, some cases showed clinical findings similar to endolymphatic hydrops. We should bear in mind that clinical Meniere’s syndrome involves ADSD. Further investigation is needed to resolve the etiology of SNHL of ADSD.     

Hearing loss in an institutionalized mentally retarded population. Identification by auditory brainstem response

We report the results of auditory brainstem response testing of 122 profoundly retarded institutionalized children, a segment of the retarded population heretofore generally regarded as untestable by behavioral audiometry. Major findings include: 32% of the study population showed, by auditory brainstem response, hearing loss exceeding 20 decibels of hearing level in one or both ears (12% showed conductive loss and 20% sensorineural loss); of the 15.6% with evidence of bilateral sensorineural loss, 7.37% had losses in the 30- to 50-dB range, and 8.19% had losses of 60 dB or greater; and evidence of abnormal brain-stem function was found in 11%. Results of otologic examinations and audiologic habilitative follow-up in selected children are also reported.     

Ototoxic impact of cisplatin in pediatric oncology patients

OBJECTIVE: To describe hearing changes in a group of 28 children (age range, 8-180 mo) undergoing protocol-based cisplatin therapy. METHODS: Conventional, play audiometry, visual reinforcement audiometry (VRA), immittance audiometry, transient click evoked otoacoustic emissions (OAEs), and auditory brainstem response (ABR) evoked potentials were used to assess peripheral sensitivity and for threshold determination. RESULTS: Bilateral symmetrical high-frequency sensorineural hearing loss was noted in 9 of the 28 children (26%). Hearing loss was evident as early as 1 month after chemotherapy and as late as 50 months and was not dependent on individual or cumulative dosage of cisplatin. CONCLUSIONS: 1) Presence of sensorineural hearing loss was independent of individual and/or cumulative dosage of cisplatin; 2) audiologic assessment should be incorporated into a child's periodic medical evaluations after chemotherapy treatment, as onset of sensorineural hearing loss cannot be predicted; 3) personal hearing aids may be indicated for those children with hearing loss affecting the low- to mid-frequencies; a personal assistive listening device (frequency modulated system) may be more appropriate for losses above 3000 Hz; and 4) evaluation and intervention by a speech-language pathologist may be indicated to address possible articulation or language development problems consequent to hearing loss.