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Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Prevention and management of hearing loss in syndromic craniosynostosis: A case series
Elisa Biamino1, Andrea Canale2, Michelangelo Lacilla2
1Department of Pediatrics, University of Torino, Torino, Italy.
Insights
Children with syndromic craniosynostosis frequently experience hearing loss, primarily due to middle ear issues like otitis media with effusion and cholesteatoma. Regular audiological monitoring is essential for early detection and management of hearing impairments in these patients.
Area of Science:
- Pediatric Otolaryngology
- Craniofacial Surgery
- Genetics and Audiology
Background:
- Syndromic craniosynostosis involves premature fusion of cranial sutures, often associated with craniofacial abnormalities.
- Audiological deficits are common in children with syndromic craniosynostosis, but the specific profiles require further elucidation.
- Understanding these profiles is crucial for timely intervention and management.
Purpose of the Study:
- To evaluate the audiological characteristics in a group of children diagnosed with syndromic craniosynostosis.
- To determine the prevalence, type, and severity of hearing loss in this cohort.
- To investigate the association between otologic diseases and hearing impairment.
Main Methods:
- A cohort of 11 children with various syndromic craniosynostosis types underwent comprehensive audiological assessments.
- Evaluations included otoscopy, pure-tone audiometry, tympanometry, acoustic reflex testing, auditory brainstem response (ABR), otoacoustic emissions, and temporal bone high-resolution computed tomography (HRCT).
- Key outcomes measured were hearing loss prevalence, type, severity, and the incidence of chronic otitis media.
Main Results:
- 64% of the children presented with hearing loss (HL), with 43% having conductive HL and 57% having mixed HL.
- No cases of purely sensorineural HL were observed.
- Middle ear disorders were prevalent in all hearing-impaired patients, including otitis media with effusion and tympanic alterations or cholesteatoma in mixed HL cases.
Conclusions:
- This study highlights a high prevalence of otologic diseases contributing to hearing impairment in children with syndromic craniosynostosis.
- Middle ear pathologies were the primary cause of hearing loss, even in cases with mixed HL.
- Consistent audiological follow-up is strongly recommended for this patient population.
Objective:
To assess the audiological profile in a cohort of children affected by syndromic craniosynostosis.
Methods:
Eleven children with Apert syndrome (n=4), Saethre-Chotzen syndrome (n=3), Muenke syndrome (n=2), Crouzon syndrome (n=1) and Pfeiffer syndrome type 1 (n=1) were submitted to a complete audiologic evaluation including otoscopy, pure-tone audiometry, tympanometry and acoustic reflex testing, ABR, otoacustic emissions, temporal bone High Resolution CT (HRCT) scan. The main outcome measures were prevalence, type and severity of hearing loss, prevalence of chronic otitis media, correlation with the time of first surgical correction.
Results:
Seven of 11 patients (64%) presented hearing loss (HL), conductive in 3/7 patients (43%) and mixed in 4/7 (57%). No patients showed a purely sensorineural HL. All hearing impaired patients displayed middle ear disorders: the patients with conductive HL had otitis media with effusion (OME) and 3/4 patients with mixed HL showed tympanic alterations or cholesteatoma. A bilateral vestibular aqueduct enlargement was detected by HRCT scan in one normal hearing patient. The ABRs resulted normal in all cases.
Conclusion:
Our study confirms the high prevalence of otologic diseases in such patients. In contrast with previous studies, middle ear disorders were responsible for the hearing impairment also in patients with mixed HL due to secondary inner ear damage. These findings restate the necessity of a close audiologic follow-up. We did not detect the specific ABR abnormalities previously reported, possibly because of an early correction of the cranial vault malformations.
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