Preserved otoacoustic emission in a child with a profound unilateral sensorineural hearing loss

Ohwatari, Ryuichiro; Fukuda, Satoshi; Chida, Eiji; Matsumura, Michiya; Kuroda, Takeo; Kashiwamura, Masaaki; Inuyama, Yukio

doi:10.1016/s0385-8146(01)00063-3

Cited by 9 publications

(8 citation statements)

References 11 publications

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“…Though well-studied in adults due to its association with acoustic neuromas, the etiology of unilateral neural hearing loss remains poorly understood in children. To our knowledge, only 10 cases have been published, and etiology was rarely identified [18][19][20]. Therefore, we studied UNHL in children, testing our hypothesis that its etiologies are different from unilateral SNHL in general.…”

Section: Discussionmentioning

confidence: 99%

Etiology of unilateral neural hearing loss in children

Laury

Casey

McKay

et al. 2009

International Journal of Pediatric Otorhinolaryngology

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Section: Discussionmentioning

confidence: 99%

Etiology of unilateral neural hearing loss in children

Laury

Casey

McKay

et al. 2009

International Journal of Pediatric Otorhinolaryngology

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“…ANs are thought to usually present bilaterally, but recent studies suggest that some AN cases involve only one ear [2–4], accounting for less than 10 percent of all ANs. The causes of unilateral AN are still unclear; however, cochlear nerve deficiency, either partially (hypoplasia) or completely (aplasia or agenesis), has recently been recognized as a significant cause of SNHL.…”

Section: Discussionmentioning

confidence: 99%

“…Most ANs show bilateral presentation accompanied by typical audiological features, but recent studies suggest that some AN cases involve only one ear [2–4]. This kind of patients could easily be missing because of the present EOAE during the newborn hearing screening.…”

Section: Introductionmentioning

confidence: 99%

Unilateral Auditory Neuropathy Caused by Cochlear Nerve Deficiency

Cheng

Bu²,

Wu³

et al. 2012

International Journal of Otolaryngology

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Objective. To explore possible corelationship between the cochlear nerve deficiency (CND) and unilateral auditory neuropathy (AN). Methods. From a database of 85 patients with unilateral profound sensorineural hearing loss, eight who presented with evoked otoacoustic emissions (EOAEs) or cochlear microphonic (CM) in the affected ear were diagnosed with unilateral AN. Audiological and radiological records in eight patients with unilateral AN were retrospectively reviewed. Results. Eight cases were diagnosed as having unilateral AN caused by CND. Seven had type “A” tympanogram with normal EOAE in both ears. The other patient had unilateral type “B” tympanogram and absent OAE but CM recorded, consistent with middle ear effusion in the affected ear. For all the ears involved in the study, auditory brainstem responses (ABRs) were either absent or responded to the maximum output and the neural responses from the cochlea were not revealed when viewed by means of the oblique sagittal MRI on the internal auditory canal. Conclusion. Cochlear nerve deficiency can be seen by electrophysiological evidence and may be a significant cause of unilateral AN. Inclined sagittal MRI of the internal auditory canal is recommended for the diagnosis of this disorder.

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“…Abnormal OAE was found in 10 ears without hearing loss, whereas it was normal in five ears with mild sensorineural‐type hearing loss (PTA 0.5–2 kHz 16.7–25 dB HL) and in one ear with conductive type hearing loss (PTA 0.5–2 kHz 30 dB HL). While normal OAE is known to be found in patients with degeneration in the acoustic nerve and preserved hair cells in the inner ear, pathological OAE in patients with normal hearing at audiometry may be caused by partly loss of hair cells in the inner ear and, therefore, predict future hearing loss [Brown and Dort, 2001; Ohwatari et al, 2001].…”

Section: Discussionmentioning

confidence: 99%

Vestibular dysfunction in adult patients with osteogenesis imperfecta

Kuurila

Kentala

Karjalainen

et al. 2003

American J of Med Genetics Pt A

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Progressive hearing loss is a major symptom in osteogenesis imperfecta (OI), a genetic brittle bone disease. Vertigo is frequently associated with otosclerosis in which the hearing loss clinically resembles that in OI. Vertigo is also common in basilar impression (BI) found in up to 25% of adult OI patients. In order to evaluate the cause, frequency, and characteristics of vertigo in OI, 42 patients were studied by interview, clinical examination, and audiological examination supplemented with electronystagmography (ENG) and lateral skull radiography. Audiometry showed hearing loss in 25 patients (59.5%). Nine patients (21%) displayed abnormal skull base anatomy in the forms of basilar impression, basilar invagination, or both, all designated here as BI. Twenty-two patients (52.4%) reported vertigo, mostly of floating or rotational sensation of short duration. Patients with hearing loss tended to have more vertigo than patients with normal hearing. Vertigo was not correlated with type of hearing loss or auditory brain-stem response (ABR) pathology. ENG was abnormal in 14 patients (33.3%). No dependency was found between vertigo and deviant ENG results. Patients with BI tended to have more vertigo than patients with normal skull base but the difference was not statistically significant. Neither ENG pathology, nor the presence or type of hearing loss showed correlation with BI. In conclusion, vertigo is common in patients with OI. In most cases, it may be secondary to inner ear pathology, and in only some patients does BI explain it. Since some OI patients without BI or hearing loss also suffer from vertigo, further clinical and neurological studies are needed to define the pathogenesis of vertigo in OI.

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Preserved otoacoustic emission in a child with a profound unilateral sensorineural hearing loss

Cited by 9 publications

References 11 publications

Etiology of unilateral neural hearing loss in children

Etiology of unilateral neural hearing loss in children

Unilateral Auditory Neuropathy Caused by Cochlear Nerve Deficiency

Vestibular dysfunction in adult patients with osteogenesis imperfecta

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