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Fulltext Congenital cholesteatoma: delayed diagnosis and its consequences

Goh BS, Faizah AR, Salina H, Asma A, Saim L

Med J Malaysia, 2010 Sep;65(3):196-8.
PMID: 21939167

This is a retrospective review of congenital cholesteatoma cases that were managed surgically. There were 5 cases. The age of presentation ranged from 5 to 18 year old. Three patients presented with complication of the disease. Three patients had intact tympanic membrane, two had perforation at the anterior superior quadrant. All patients had cholesteatoma medial to tympanic membrane. Four cases had extensive ossicular erosion with preoperative hearing worse than 40 dB. Four cases underwent canal wall down mastoid surgery and one underwent canal wall up surgery. One patient had recurrence which required revision surgery. In conclusion, congenital cholesteatoma presented late due to the silent nature of disease in its early stage. Extensive disease, ossicular destruction with risk of complication at presentation were marked in our study. Hence, more aggressive surgical intervention is recommended in the management of congenital cholesteatoma.

Matched MeSH terms: Hearing Loss/etiology*
Risk factors associated with hearing loss in term neonates with hyperbilirubinaemia

Boo NY, Oakes M, Lye MS, Said H

J Trop Pediatr, 1994 Aug;40(4):194-7.
PMID: 7932930

A study of 128 jaundiced term neonates showed that 28 (22 per cent) had hearing loss based on brain stem-evoked response. There was no significant difference in the percentage of neonates with hearing loss between those with peak serum bilirubin levels of less than 340 mumol/l (16 per cent) and those with hyperbilirubinaemia > 339 mumol/l (33 per cent) (P = 0.11). Logistic regression analysis showed that severe jaundice which required exchange transfusion and earlier age of onset of hyperbilirubinaemia were statistically significant risk factors associated with hearing loss (P = 0.038 and P = 0.012, respectively).

Matched MeSH terms: Hearing Loss/etiology*
Histopathology of the inner ear in unoperated acoustic neuroma

Mahmud MR, Khan AM, Nadol JB

Ann Otol Rhinol Laryngol, 2003 Nov;112(11):979-86.
PMID: 14653368

Although hearing loss is the most common presenting symptom in patients with acoustic neuroma, the pathophysiology of hearing loss associated with acoustic neuroma is unknown. Although primary dysfunction of the auditory nerve is intuitively logical, available histopathologic and clinical data suggest that although neural degeneration is common, it alone does not adequately account for hearing loss in many cases. The purpose of this study was to evaluate 11 cases of unoperated unilateral acoustic neuromas. Temporal bones were identified by means of a search mechanism provided by the National Temporal Bone, Hearing, and Balance Pathology Resource Registry and were prepared for light microscopy by standard techniques. Quantification of spiral ganglion cells, hair cells, stria vascularis, and spiral ligament was accomplished for each specimen. In addition, the maximum diameter and volume of each tumor were calculated from histopathologic sections. Increasing tumor size did predict a reduced spiral ganglion count. However, although there was a tendency for decreasing spiral ganglion cell count and for increasing tumor size to predict a higher pure tone average and lower speech discrimination score, these correlations did not reach statistical significance. In tumor ears in which the speech discrimination score was 50% or less, there was always significant degeneration of other structures of the inner ear in addition to neurons, including hair cells, the stria vascularis, and the spiral ligament. Endolymphatic hydrops and eosinophilic precipitate in the perilymphatic spaces were found in 2 of 3 such cases. It is concluded that acoustic neuromas appear to cause hearing loss, not only by causing degeneration of the auditory nerve, but also by inducing degenerative changes in the inner ear. It is hypothesized that the proteinaceous material seen histologically may represent the products of up-regulated genes in acoustic neuroma, some of which may interfere with normal cochlear function.

Matched MeSH terms: Hearing Loss/etiology
Mother with post-partum group B Streptococcus meningitis and cerebellar abscess

Ghani NA, Jaafar R, Ishak S, Zainuddin AA, Mukari SA, Mahdy ZA

J Obstet Gynaecol Res, 2007 Apr;33(2):195-8.
PMID: 17441895

We report the case of a 25-year-old Malay woman, admitted for preterm delivery at 35 weeks' gestation. Vaginal swab did not isolate any organism. She delivered a baby girl who developed respiratory distress syndrome, requiring ventilation. Although chest radiograph showed hyaline membrane disease with pneumonia, septic workout was negative. The mother was discharged on the next day. Seven days postpartum, the mother presented with fever and fits and was diagnosed to have meningo-encephalitis. Lumbar puncture isolated group B Streptococcus (GBS) and MRI revealed a superior cerebellar abscess. She was treated and survived the episode. This case illustrates the uncommon situation where GBS infection was confirmed via maternal septic workout rather than neonatal, although both presented with severe disease.

Matched MeSH terms: Hearing Loss/etiology
A study of the results of speech language and hearing assessment of three groups of repaired cleft palate children and adults

Abdullah S

Ann Acad Med Singap, 1988 Jul;17(3):388-91.
PMID: 3218930

The results of speech, language and hearing assessment of repaired cleft palate children and adults over a 4-year period at the Speech/Language therapy unit, Department of ENT, Faculty of Medicine, UKM are reported. The subjects were 33 incomplete cleft lip palate (ICLP), 48 unilateral complete cleft lip and palate (UCLP) and 26 bilateral complete cleft lip and palate (BCLP). Results obtained for the following assessments are reported and discussed: (i) Hearing assessment, (ii) intelligibility rate, (iii) severity of hyper-nasality and (iv) types of articulation errors and school/behaviour problems and nasal grimace. Due to subject variability in ethnicity, language and dialects and the lack of normal data, it is difficult to compare or repeat previously reported results. Assessment of intelligibility, hypernasality and articulation were conducted using the Malay language. Results obtained were compared among the three groups of subjects. The significant findings were that hypernasality, intelligibility and articulation errors were more severe in the ICLP group than in the UCLP or BCLP groups of patients. This is contrary to expectations and cannot be explained in terms of the type and/or the degree of clefting. Hence, special attention should be paid to the less overt type of cleft patients in so far as their speech assessment and rehabilitation are concerned.

Matched MeSH terms: Hearing Loss/etiology*
An audiological evaluation of syndromic and non-syndromic craniosynostosis in pre-school going children

Goh LC, Azman A, Siti HBK, Khoo WV, Muthukumarasamy PA, Thong MK, et al.

Int J Pediatr Otorhinolaryngol, 2018 Jun;109:50-53.
PMID: 29728184 DOI: 10.1016/j.ijporl.2018.03.010

OBJECTIVE: To study the audiological outcome and early screening of pre-school going children with craniosynostosis under follow-up at the University of Malaya Medical Center(UMMC), Kuala Lumpur, Malaysia over a 10 year period.
METHODS: A retrospective descriptive cohort study on the audiological findings detected during the first hearing assessment done on a child with craniosynostosis using otoacoustic emissions, pure tone audiometry or auditory brainstem response examination. The main aim of this study was to evaluate the type and severity of hearing loss when compared between syndromic and non-sydromic craniosynostosis, and other associated contributory factors.
RESULTS: A total of 31 patients with 62 ears consisting of 14 male patients and 17 female patients were evaluated. Twenty two patients (71%) were syndromic and 9 (29%) were non-syndromic craniosynostosis. Amongst the syndromic craniosynostosis, 9 (41%) had Apert syndrome, 7 (32%) had Crouzon syndrome, 5 (23%) had Pfieffer syndrome and 1 (4%) had Shaethre Chotzen syndrome. Patients with syndromic craniosynostosis were more likely to present with all types and severity of hearing loss, including severe to profound sensorineural hearing loss while children with non-syndromic craniosynostosis were likely to present with normal hearing (p loss including sensorineural hearing loss is more likely to be present in a child with syndromic craniosynostosis (p loss, including that of a severe to profound degree compared to children with non-syndromic craniosynostosis. In addition to that, hearing loss is more likely to be detected when the first hearing test is done at a later age, and this can be an irreversible sensorineural hearing loss. We would like to advocate the need for early audiological screening and follow up in children with syndromic craniosynostosis.

Matched MeSH terms: Hearing Loss/etiology*
Fulltext Acoustic neuroma with orofacial paresthesia: description of an atypical presentation

Lim CC, Misron K, Liew YT, Wong EHC

BMJ Case Rep, 2019 Nov 04;12(11).
PMID: 31690691 DOI: 10.1136/bcr-2019-232275

Acoustic neuroma (AN) usually manifests with asymmetric hearing loss, tinnitus, dizziness and sense of disequilibrium. About 10% of patients complain of atypical symptoms, which include facial numbness or pain and sudden onset of hearing loss. Patients with atypical symptoms also tend to have larger tumours due to the delay in investigation. We report a particularly interesting case of a patient presented to us with numbness over her right hemifacial region after a dental procedure without significant acoustic and vestibular symptoms. Physical examination and pure tone audiometry revealed no significant findings but further imaging revealed a cerebellopontine angle mass. The changing trends with easier access to further imaging indicate that the presentation of patients with AN are also changing. Atypical symptoms which are persistent should raise clinical suspicion of this pathology among clinicians.

Matched MeSH terms: Hearing Loss/etiology
Fulltext Research on Hygiene Based on Fieldwork and Experimental Studies

Yajima I

Nihon Eiseigaku Zasshi, 2017;72(1):49-54.
PMID: 28154361 DOI: 10.1265/jjh.72.49

Several experimental studies on hygiene have recently been performed and fieldwork studies are also important and essential tools. However, the implementation of experimental studies is insufficient compared with that of fieldwork studies on hygiene. Here, we show our well-balanced implementation of both fieldwork and experimental studies of toxic-element-mediated diseases including skin cancer and hearing loss. Since the pollution of drinking well water by toxic elements induces various diseases including skin cancer, we performed both fieldwork and experimental studies to determine the levels of toxic elements and the mechanisms behind the development of toxic-element-related diseases and to develop a novel remediation system. Our fieldwork studies in several countries including Bangladesh, Vietnam and Malaysia demonstrated that drinking well water was polluted with high concentrations of several toxic elements including arsenic, barium, iron and manganese. Our experimental studies using the data from our fieldwork studies demonstrated that these toxic elements caused skin cancer and hearing loss. Further experimental studies resulted in the development of a novel remediation system that adsorbs toxic elements from polluted drinking water. A well-balanced implementation of both fieldwork and experimental studies is important for the prediction, prevention and therapy of toxic-element-mediated diseases.

Matched MeSH terms: Hearing Loss/etiology
Cholesteatoma in patients with congenital external auditory canal anomalies: retrospective review

Mazita A, Zabri M, Aneeza WH, Asma A, Saim L

J Laryngol Otol, 2011 Nov;125(11):1116-20.
PMID: 21846418 DOI: 10.1017/S0022215111002052

To review cases of congenital external auditory canal anomaly with cholesteatoma, documenting clinical presentation, cholesteatoma site and extent, complications, and surgery.

Matched MeSH terms: Hearing Loss/etiology
Hearing impairment and contributing factors among fertilizer factory workers

Saffree Jeffree M, Ismail N, Awang Lukman K

J Occup Health, 2016 Sep 30;58(5):434-443.
PMID: 27488035

INTRODUCTION: Hearing impairment remains the main occupational health problem in the manufacturing industry, and its contributing factors have not been well controlled.
METHODS: Unmatched case control and comparative studies were carried out among fertilizer factory workers in Sarawak with the aim of determining contributing factors for hearing impairment. Respondents consisted of 49 cases that were diagnosed from 2005 to 2008 with 98 controls from the same work places. Chi-square test and Mann-Whitney test were used in a univariate analysis to determine the association between hearing impairment and the contributing risks being studied.
RESULTS: The results of the univariate analysis showed that hearing impairment was significantly (p<0.05) associated with older age, lower education level, high smoking dose, high occupational daily noise dose, longer duration of service, infrequent used of hearing protection device (HPD), and low perception of sound on HPD usage. Multivariate logistic regression of hearing impairment after controlling for age found the following five variables: occupational daily noise dose ≥50% (OR 3.48, 95% CI 1.36-8.89), ≥15 years of services (OR 2.92, 95% CI 1.16-7.33), infrequent use of HPD (OR 2.79, 95% CI 1.15-6.77), low perception of sound on HPD (POR 2.77, 95% CI 1.09-6.97), and smoking more than 20 packs per year (OR 4.71, 95% CI 1.13-19.68).
DISCUSSION: In conclusion, high occupational noise exposure level, longer duration of service, low perception of sound on HPD, infrequent used of HPD, and smoking more than 20 packs per year were the contributing factors to hearing impairment, and appropriate intervention measures should be proposed and taken into considerations.

Matched MeSH terms: Hearing Loss/etiology*