Sensorineural hearing loss in pediatric patients with celiac disease

2014 ◽  
Vol 272 (9) ◽  
pp. 2149-2151 ◽  
Author(s):  
Nafiye Urganci ◽  
Derya Kalyoncu ◽  
Asli Batur Calis
2009 ◽  
Vol 23 (8) ◽  
pp. 531-535 ◽  
Author(s):  
Umberto Volta ◽  
Gian Gaetano Ferri ◽  
Roberto De Giorgio ◽  
Angela Fabbri ◽  
Claudia Parisi ◽  
...  

BACKGROUND: Celiac disease (CD) can be associated with a variety of extraintestinal manifestations, including neurological diseases. A new neurological correlation has been found between CD and sensorineural hearing loss (SNHL).OBJECTIVE: To verify the association between SNHL and CD, and to establish whether the neurological hearing impairment in CD is related to nonorgan-specific and antineuronal antibodies, as well as the presence of autoimmune disorders.METHODS: A sample of 59 consecutive biopsy- and serologically proven CD patients were studied. Among CD patients, 11 were newly diagnosed and 48 were on a gluten-free diet. Hearing function was assessed by audiometric analysis in all CD patients as well as in 59 age- and sex-matched controls. Patients were tested for a panel of immune markers including nonorgan-specific autoantibodies and antineuronal antibodies.RESULTS: SNHL was detected in five CD patients (8.5%) and in two controls (3.4%). In one patient, the SNHL was bilateral, whereas the remaining four had a monolateral impairment. The prevalence of SNHL was not significantly different between CD patients and controls. At least one of the antibodies tested for was positive in two of the five CD patients with SNHL and in 12 of the 54 CD patients without SNHL. Antineuronal antibodies to central nervous system antigens were consistently negative in the five CD patients with SNHL. Only one of the five CD patients with SNHL had Hashimoto thyroiditis.CONCLUSIONS: SNHL and CD occur coincidentally. Hearing function should be assessed only in CD patients with clinical signs of hearing deficiency.


2020 ◽  
pp. 014556132097260
Author(s):  
Dilhara Karunaratne ◽  
Nisal Karunaratne

Objectives: Celiac disease is a common multisystemic autoimmune disorder. It is now increasingly recognized that it may present with extraintestinal manifestations which contribute to the difficulty in its diagnosis. The objective of this scholarly review was to examine the extraintestinal ENT manifestations of celiac disease and its pathophysiology and management, in order to highlight that some patients with celiac disease may present initially to the otolaryngologist. Improving awareness of celiac disease among otolaryngologists may aid in the correct diagnosis and correct management plan. Methods: A literature review was conducted using the PubMed database to identify original articles related to celiac disease and ENT manifestations between the years 2000 and 2020. The search was performed using the search string: (“coeliac disease” OR “celiac disease”) AND (“ENT manifestations” OR “hearing loss” OR “epistaxis” OR “nasal septal perforation” OR “obstructive sleep apnoea” OR “vertigo” OR “tonsillitis” OR “sinusitis”). Only articles written in English were reviewed. Results: A total of 17 papers met the inclusion criteria. Extraintestinal ENT manifestations of celiac disease include sensorineural hearing loss, obstructive sleep apnea, nasal septal perforation, epistaxis, and vertigo with nystagmus. Sensorineural hearing loss, obstructive sleep apnea, nasal septal perforation, vertigo, and nystagmus are thought to result from immunologically mediated mechanisms, with intestinal malabsorption resulting in epistaxis. Conclusions: Celiac disease can cause extraintestinal ENT manifestations and requires a high index of suspicion from the otolaryngologist to diagnose and suitably manage. A gluten-free diet may result in sufficient symptom resolution for most manifestations. Sensorineural hearing loss due to celiac disease appears to be progressive and permanent and may require frequent audiological monitoring.


2019 ◽  
Vol 30 (9) ◽  
pp. 776-781 ◽  
Author(s):  
Alper Yazici ◽  
◽  
Abdullah Emre Yildirim ◽  
Bugra Tolga Konduk ◽  
◽  
...  

2012 ◽  
Vol 147 (5) ◽  
pp. 932-936 ◽  
Author(s):  
Peter J. King ◽  
Xiaomei Ouyang ◽  
Lilin Du ◽  
Denise Yan ◽  
Simon I. Angeli ◽  
...  

Objectives Determine the diagnostic yield of a shared genetic testing algorithm in adult and pediatric populations with sensorineural hearing loss (SNHL) and recommend effective testing strategies to evaluate for genetic causes of deafness in patients presenting with idiopathic sensorineural hearing loss. Study Design Hospital-based cohort study. Setting University of Miami outpatient otology clinics between 2001 and 2010. Subjects Two hundred twenty-one adult and 163 pediatric patients with nonsyndromic sensorineural hearing loss. Methods Peripheral blood samples were screened for mutations in GJB2 and GJB6 and mitochondrial DNA mutations 1555A>G, 7444G>A, and 3243A>G. Audiometric data and family history were also collected. Results GJB2/ GJB6-related deafness was diagnosed in 23 of 163 pediatric patients (14%) compared with only 3 of 221 adults (1%). All adults had a family history of hearing loss, and 2 patients noted deafness onset at birth. Nineteen GJB2 mutations were identified with 35delG the most common mutation. The 35delG homozygous state was the most common pathogenic genotype (54%). Mitochondrial DNA (mtDNA) mutations were found in 6 adult probands (3%). No mtDNA mutations were found in pediatric patients. Conclusion Testing for common GJB2/ GJB6 mutations in pediatric patients has considerable value in establishing an etiologic diagnosis for SNHL. Similar testing in adults is of very low yield except perhaps in cases of early-onset SNHL or strong family history. Mitochondrial DNA testing should be considered in adults with idiopathic SNHL.


2005 ◽  
Vol 63 (4) ◽  
pp. 969-971 ◽  
Author(s):  
Emerson L. Gasparetto ◽  
Arnolfo de Carvalho Neto ◽  
Danny Warszawiak ◽  
Isac Bruck ◽  
Sérgio Antoniuk ◽  
...  

OBJECTIVE: To describe the MRI findings of two pediatric patients with sudden sensorineural hearing loss (SSHL). CASE REPORTS: Two male patients (two-year and three-months-old, and one year and four-months-old) presented with sudden dumbness. Physical and neurological examinations were unremarkable besides bilateral hypoacusia. All the laboratory investigation was negative, and brain stem auditory evoked potentials showed deep bilateral deafness in both cases. MRI studies revealed normal inner ears and multifocal white matter areas of slight low signal on T1-weighted images and high signal on FLAIR images. The follow-up MRI studies and neurological examinations did not demonstrate alterations in the previous findings. CONCLUSION: Pediatric patients with SSHL may present cerebral white matter signal abnormalities at the MRI as the only finding. Further studies with larger casuistics need to be conducted to elucidate these findings.


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