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Inner ear anomalies and conductive hearing loss in children with Apert syndrome: an overlooked otologic aspect.
Otol Neurotol. 2009 Feb; 30(2):184-9.ON

Abstract

OBJECTIVE

To identify the occurrence of inner ear structural anomalies and conductive hearing loss (CHL) in children with Apert syndrome.

STUDY DESIGN

Retrospective review.

SETTING

Pediatric tertiary referral center.

PATIENTS

Twenty pediatric patients with Apert syndrome were found; all patients (38/40 ears) had inner ear anomalies.

INTERVENTION(S)

Computerized tomography of the head/temporal bone, pure-tone (including air and bone conduction) audiometry, and tympanometry.

MAIN OUTCOME MEASURE(S)

Imaging demonstrating inner ear anomalies, including malformations of the cochlea, dilated vestibule, and/or semicircular canal; audiologic findings of air-bone gap(s).

RESULTS

Hearing loss was found in 90% of the patients with Apert syndrome, and 80% of them had CHL. Air-bone gaps were found at all frequencies, with larger gaps at low frequencies. Fifty percent (20/40) of the ears had better than 0 dB hearing level bone conduction thresholds at 250 and/or 500 Hz. Normal middle ear pressure and mobility were found in all ears with intact eardrum. Inner ear anomalies were found in all patients, and 90% of them had bilateral involvement. Most frequently observed inner ear anomalies were dilated vestibule, malformed lateral semicircular canal, and cochlear dysplasia.

CONCLUSION

Children with Apert syndrome may present with significant CHL that cannot be explained by minor middle ear pathologies alone. This conductive loss may be, at least partially, attributed to the inner ear anomalies; however, these structural anomalies are usually not recognized in these patients. Failure to close air-bone gap after surgical intervention may raise the suspicion of inner ear anomalies, and computed tomographic scan of the temporal bone can provide definitive proof.

Authors+Show Affiliations

Department of Otolaryngology and Communication Enhancement, Children's Hospital Boston, Boston, Massachusetts 02115, USA. guangwei.zhou@childrens.harvard.eduNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

19169132

Citation

Zhou, Guangwei, et al. "Inner Ear Anomalies and Conductive Hearing Loss in Children With Apert Syndrome: an Overlooked Otologic Aspect." Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, vol. 30, no. 2, 2009, pp. 184-9.
Zhou G, Schwartz LT, Gopen Q. Inner ear anomalies and conductive hearing loss in children with Apert syndrome: an overlooked otologic aspect. Otol Neurotol. 2009;30(2):184-9.
Zhou, G., Schwartz, L. T., & Gopen, Q. (2009). Inner ear anomalies and conductive hearing loss in children with Apert syndrome: an overlooked otologic aspect. Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, 30(2), 184-9. https://doi.org/10.1097/MAO.Ob013e318191a352
Zhou G, Schwartz LT, Gopen Q. Inner Ear Anomalies and Conductive Hearing Loss in Children With Apert Syndrome: an Overlooked Otologic Aspect. Otol Neurotol. 2009;30(2):184-9. PubMed PMID: 19169132.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Inner ear anomalies and conductive hearing loss in children with Apert syndrome: an overlooked otologic aspect. AU - Zhou,Guangwei, AU - Schwartz,Lynn Thomas, AU - Gopen,Quinton, PY - 2009/1/27/entrez PY - 2009/1/27/pubmed PY - 2009/4/17/medline SP - 184 EP - 9 JF - Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology JO - Otol Neurotol VL - 30 IS - 2 N2 - OBJECTIVE: To identify the occurrence of inner ear structural anomalies and conductive hearing loss (CHL) in children with Apert syndrome. STUDY DESIGN: Retrospective review. SETTING: Pediatric tertiary referral center. PATIENTS: Twenty pediatric patients with Apert syndrome were found; all patients (38/40 ears) had inner ear anomalies. INTERVENTION(S): Computerized tomography of the head/temporal bone, pure-tone (including air and bone conduction) audiometry, and tympanometry. MAIN OUTCOME MEASURE(S): Imaging demonstrating inner ear anomalies, including malformations of the cochlea, dilated vestibule, and/or semicircular canal; audiologic findings of air-bone gap(s). RESULTS: Hearing loss was found in 90% of the patients with Apert syndrome, and 80% of them had CHL. Air-bone gaps were found at all frequencies, with larger gaps at low frequencies. Fifty percent (20/40) of the ears had better than 0 dB hearing level bone conduction thresholds at 250 and/or 500 Hz. Normal middle ear pressure and mobility were found in all ears with intact eardrum. Inner ear anomalies were found in all patients, and 90% of them had bilateral involvement. Most frequently observed inner ear anomalies were dilated vestibule, malformed lateral semicircular canal, and cochlear dysplasia. CONCLUSION: Children with Apert syndrome may present with significant CHL that cannot be explained by minor middle ear pathologies alone. This conductive loss may be, at least partially, attributed to the inner ear anomalies; however, these structural anomalies are usually not recognized in these patients. Failure to close air-bone gap after surgical intervention may raise the suspicion of inner ear anomalies, and computed tomographic scan of the temporal bone can provide definitive proof. SN - 1537-4505 UR - https://www.unboundmedicine.com/medline/citation/19169132/Inner_ear_anomalies_and_conductive_hearing_loss_in_children_with_Apert_syndrome:_an_overlooked_otologic_aspect_ L2 - https://doi.org/10.1097/MAO.Ob013e318191a352 DB - PRIME DP - Unbound Medicine ER -