Middle Ear Fibrosis Contributes to Hearing Loss in Patients With Myhre Syndrome.
| Autor: | Tayara A; School of Medicine, University of Mississippi Medical Center, Jackson, MS, USA., Hendon LG; Division of Medical Genetics, Department of Pediatrics and OBGYN, University of Mississippi Medical Center, Jackson, MS, USA., Barrera SC; Department of Otolaryngology and Communicative Sciences, University of Mississippi Medical Center, Jackson, MS, USA., Carron JD; Department of Otolaryngology and Communicative Sciences, University of Mississippi Medical Center, Jackson, MS, USA. |
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| Jazyk: | angličtina |
| Zdroj: | Ear, nose, & throat journal [Ear Nose Throat J] 2024 Jun 22, pp. 1455613241256474. Date of Electronic Publication: 2024 Jun 22. |
| DOI: | 10.1177/01455613241256474 |
| Abstrakt: | Myhre syndrome (MS) is a rare genetic condition that presents with multiple genetic anomalies including cleft lip and palate and Eustachian tube dysfunction. These patients are at a high risk for airway scarring from intubation and mucosal inflammation. Hearing loss (conductive or mixed, of varying severity) is a common comorbidity in these patients, the exact etiology of which is still unclear. We present the cases of 2 unrelated children with MS who suffered progressive mixed hearing loss from fibrosis and obliteration of the middle ear spaces. Both patients had multiple sets of ear tubes that demonstrated early extrusion. The older patient underwent bone conduction implantation at age 11 which resulted in dramatic improvement of speech recognition and interactive skills. The other younger patient demonstrates a similar trajectory but has not yet undergone implantation. Otolaryngologists should take a cautious approach to surgery of the eardrum and middle ear to avoid unnecessary induction of fibrosis in this susceptible patient population. These cases highlight a newly described etiology for hearing loss and suggest a benefit to bone conduction implantation. Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. |
| Databáze: | MEDLINE |
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