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Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Audiologic findings in Saethre-Chotzen syndrome
Heather Rosen1, Brian T Andrews, John G Meara
1Boston, Mass. From the Departments of Plastic and Oral Surgery and Genetics, Children's Hospital Boston.
Plastic and Reconstructive Surgery
|May 3, 2011
Summary
Most children with Saethre-Chotzen syndrome experience temporary, mild hearing loss, often linked to middle ear issues. This hearing deficit typically resolves, highlighting the need for careful audiologic evaluation.
Area of Science:
- Otolaryngology
- Genetics
- Audiology
Background:
- Hearing loss is known in Apert syndrome but less studied in other craniosynostosis disorders.
- Saethre-Chotzen syndrome is a craniosynostosis disorder with limited documentation of hearing impairment.
- Understanding hearing loss in Saethre-Chotzen syndrome is crucial for comprehensive patient care.
Purpose of the Study:
- To determine the incidence, type, and severity of hearing loss in patients with Saethre-Chotzen syndrome.
- To characterize the audiological profile of individuals diagnosed with Saethre-Chotzen syndrome.
- To investigate the transient or persistent nature of hearing deficits in this population.
Main Methods:
- Retrospective review of audiological and otological records for patients with Saethre-Chotzen syndrome.
- Inclusion criteria focused on patients with documented audiological examinations.
- Hearing loss classification followed American Speech-Language-Hearing Association guidelines.
Main Results:
- Out of 29 patients, 17 (59%) had at least one abnormal audiogram, mostly mild deficits.
- Sensorineural hearing loss was identified in 8 patients, with 5 cases resolving.
- Conductive hearing loss was noted in 6 patients, with 4 showing normal hearing on follow-up; 72% had normal hearing on final evaluation.
Conclusions:
- The majority of patients with Saethre-Chotzen syndrome experience hearing loss during childhood, typically mild.
- Hearing deficits are often associated with middle ear abnormalities and eustachian tube dysfunction, and usually resolve.
- Early misdiagnosis of hearing loss type (conductive/mixed as sensorineural) was common, emphasizing the need for serial audiological assessments.
