Audiologic findings in Saethre-Chotzen syndrome

Heather Rosen; Brian T Andrews; John G Meara; Joan M Stoler; John B Mulliken; Gary F Rogers

doi:10.1097/PRS.0b013e31820cf16a

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Audiologic findings in Saethre-Chotzen syndrome

Journal article

Peer reviewed

Audiologic findings in Saethre-Chotzen syndrome

Heather Rosen, Brian T Andrews, John G Meara, Joan M Stoler, John B Mulliken and Gary F Rogers

Plastic and reconstructive surgery (1963), Vol.127(5), pp.2014-2020

05/2011

DOI: 10.1097/PRS.0b013e31820cf16a

PMID: 21532428

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Abstract

Hearing loss has been described in Apert syndrome but is poorly documented in other craniosynostosis disorders. The authors retrospectively reviewed the audiologic and otologic records of patients with Saethre-Chotzen syndrome to define the incidence, type, and extent of hearing loss. Only patients with documented audiologic examinations were included. Hearing loss was categorized by American Speech-Language-Hearing Association guidelines (i.e., mild, 26 to 40 dB; moderate, 41 to 55 dB; moderate/severe, 56 to 70 dB; severe, 71 to 90 dB; and profound, >90 dB). Twenty-nine patients met inclusion criteria. Mean age at initial audiologic evaluation was 6.7 years (range, 0.7 to 24.5 years). Seventeen patients (59 percent) had at least one abnormal audiogram; in 15 patients, the deficit was mild. Eight patients demonstrated sensorineural hearing loss. Five cases resolved and, thus, had been mischaracterized. Six patients had conductive hearing loss on at least one examination; follow-up testing in four patients revealed normal hearing. Two patients had unspecified hearing loss by sound field method. One patient had mixed hearing loss on consecutive audiograms. Twenty-one patients (72 percent) had normal hearing on their last audiogram. Most patients with Saethre-Chotzen syndrome had hearing loss at some point during childhood. This was typically mild and correlated with middle ear abnormality and eustachian tube dysfunction. Usually, the hearing deficit resolved. Early mischaracterization of mixed hearing loss or conductive hearing loss as sensorineural hearing loss was common.

Acoustic Impedance Tests

Acrocephalosyndactylia - complications

Acrocephalosyndactylia - physiopathology

Adolescent

Adult

Audiometry - methods

Child

Child, Preschool

Diagnosis, Differential

Disease Progression

Female

Follow-Up Studies

Hearing - physiology

Hearing Loss, Sensorineural - diagnosis

Hearing Loss, Sensorineural - etiology

Hearing Loss, Sensorineural - physiopathology

Humans

Infant

Male

Prognosis

Retrospective Studies

Severity of Illness Index

Young Adult

Details

Title: Subtitle: Audiologic findings in Saethre-Chotzen syndrome
Creators: Heather Rosen - Boston Children's Hospital
Brian T Andrews
John G Meara
Joan M Stoler
John B Mulliken
Gary F Rogers
Resource Type: Journal article
Publication Details: Plastic and reconstructive surgery (1963), Vol.127(5), pp.2014-2020
DOI: 10.1097/PRS.0b013e31820cf16a
PMID: 21532428
NLM abbreviation: Plast Reconstr Surg
ISSN: 0032-1052
eISSN: 1529-4242
Language: English
Date published: 05/2011
Academic Unit: Craniofacial Anomalies Research Center; Neurosurgery; Otolaryngology
Record Identifier: 9984366281302771

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