Pawina Jiramongkolchai1, Manvinder S Kumar2, Sivakumar Chinnadurai3, Christopher T Wootten3, Steven L Goudy4. 1. Department of Otolaryngology, Washington University School of Medicine in Saint Louis, 660 South Euclid Avenue, Box 8115, St. Louis, MO, 63110, USA. 2. Emory University School of Medicine, 100 Woodruff Circle, Atlanta, GA, 30322, USA. 3. Department of Otolaryngology, Vanderbilt University Medical Center, 1215 21st Avenue South, Medical Center East, South Tower, Nashville, TN, 37232, USA. 4. Department of Otolaryngology - Head & Neck Surgery, Emory University School of Medicine, 2015 Uppergate Drive, Room 222, Atlanta, GA, 30322, USA. Electronic address: steven.goudy@emory.edu.
Abstract
OBJECTIVE: The purpose of this study was to determine the prevalence and characterize the types of hearing loss in pediatric patients with 22q11.2 deletion syndrome (22q11DS). METHODS: Fifty-eight patients were identified via retrospective chart review performed of patients with 22q11DS between 1996 and 2014. Patient demographics, pertinent family history, associated comorbidities, and degree and type of hearing loss were gathered for each patient. A literature review of the National Library of Medicine's database with a focus on hearing loss and 22q11DS was performed. RESULTS: 22 patients (38%) were found to have hearing impairment: 68% with conductive hearing loss, 14% with sensorineural hearing loss, and 18% with mixed hearing loss. Patients with hearing loss regardless of type had a higher prevalence of developmental delay (55%), cleft palate (23%), articulation disorders (77%), and a greater need for tympanostomy tubes (73%) compared to patients with normal hearing. Temporal bone computed tomography scans of 5 patients revealed a variety of abnormalities in the middle and/or inner ears. CONCLUSION: Hearing impairment occurs in up to 38% of 22q11DS patients of both conductive and sensorineural types, with the conductive type being the most common. These patients have a greater need for tympanostomy tubes and a higher prevalence of developmental delay and speech articulation disorders. Early hearing screening and treatment is warranted in this population.
OBJECTIVE: The purpose of this study was to determine the prevalence and characterize the types of hearing loss in pediatric patients with 22q11.2 deletion syndrome (22q11DS). METHODS: Fifty-eight patients were identified via retrospective chart review performed of patients with 22q11DS between 1996 and 2014. Patient demographics, pertinent family history, associated comorbidities, and degree and type of hearing loss were gathered for each patient. A literature review of the National Library of Medicine's database with a focus on hearing loss and 22q11DS was performed. RESULTS: 22 patients (38%) were found to have hearing impairment: 68% with conductive hearing loss, 14% with sensorineural hearing loss, and 18% with mixed hearing loss. Patients with hearing loss regardless of type had a higher prevalence of developmental delay (55%), cleft palate (23%), articulation disorders (77%), and a greater need for tympanostomy tubes (73%) compared to patients with normal hearing. Temporal bone computed tomography scans of 5 patients revealed a variety of abnormalities in the middle and/or inner ears. CONCLUSION:Hearing impairment occurs in up to 38% of 22q11DS patients of both conductive and sensorineural types, with the conductive type being the most common. These patients have a greater need for tympanostomy tubes and a higher prevalence of developmental delay and speech articulation disorders. Early hearing screening and treatment is warranted in this population.
Authors: E Verheij; A L Kist; A B Mink van der Molen; I Stegeman; G A van Zanten; W Grolman; H G X M Thomeer Journal: Eur Arch Otorhinolaryngol Date: 2016-11-11 Impact factor: 2.503
Authors: Kit Melissa Larsen; Giovanni Pellegrino; Michelle Rosgaard Birknow; Trine Nørgaard Kjær; William Frans Christiaan Baaré; Michael Didriksen; Line Olsen; Thomas Werge; Morten Mørup; Hartwig Roman Siebner Journal: Schizophr Bull Date: 2018-02-15 Impact factor: 9.306