Cochlear implantation in older adults: 15 years' experience of a tertiary centre in a developing country.

INTRODUCTION: Cochlear implant (CI) is a viable option of treatment for older patients with severe to profound deafness in resource-rich countries. Implantation is limited in developing countries. OBJECTIVE: To review factors and outcomes of elderly patients that underwent CI in a pioneer centre in a developing country. MATERIAL AND METHODS: An observational retrospective review of patients older than 60, implanted between 2005 to 2020. RESULTS: Eleven patients were included. Patient were aged 60-74 years old with median of 66 years old. Average duration of deafness prior to implantation is 22 years. All implantation was unilateral except for one case that was implanted sequentially after 5 years Three patients were privately funded. Analysis of the hearing aided level with CI and hearing aid showed substantial improvement provided by the CI. The Categories of Auditory performance (CAP-II) scale were in the range of 6-9. DISCUSSION: Cochlear implant is safe and stable intervention in providing improvement of hearing and self-esteem in the elderly patients. Social isolation and depression also improved with better hearing and communication. CONCLUSION: Awareness of the CI in elderly patients must be advocated among policy- makers, clinicians, and patients to mitigate the clinical and public health burden of hearing loss among older patients.

Cochlear implantation outcomes in children with multiple disabilities: a topic that's worth revisiting.

OBJECTIVES: To determine the benefits of cochlear implantation in hearing loss children with multiple disabilities (MD) in terms of auditory outcomes, speech performance, and their quality of life. METHODS: This was a cross sectional study from January 2019 to December 2020 in which thirty-one children with hearing loss and multiple disabilities were evaluated. Their improvement in auditory and speech performances were assessed using Categories of Auditory Performance version II (CAP-II) and the Speech Intelligibility Rating (SIR) scales. The assessment was done at 6-month intervals, with the baseline evaluation done at least six months after activation of the implant. Parents were asked to fill the Parents Evaluation of Aural/Oral Performance of Children (PEACH) diary and Perceived Benefit Questionnaire (PBQ) to evaluate the child's quality of life. RESULTS: All 31 children have Global Developmental Delay (GDD), with 11 having an additional disability. Both mean CAP-II and SIR scores showed significant improvement with increased hearing age (p < 0.05) after 6-month intervals. In addition, 20 out of 31 children (64.5%) have achieved verbal communication after implantation. The mean PEACH score in quiet was significantly better than in noise (p = 0.007) and improved with the increased of hearing age. The majority of parents (96%‒100%) perceived a cochlear implant as beneficial to their child in terms of auditory response, awareness, interaction, communication, and speech development. CONCLUSIONS: Cochlear implantation had shown benefits in children with multiple disabilities. Outcome measures should not only focus on auditory and speech performances but the improvement in quality of life. Hence, individualized each case with realistic expectation from families must be emphasized in this group of children. LEVEL OF EVIDENCE: Level 3.

Recurrent Cholesteatoma: Why it occurs?

A cholesteatoma is an expansion of keratinizing squamous epithelium that enters the middle ear cleft from the outer layer of the tympanic membrane or ear canal. Choleatomas are always treated surgically. Recurrence of the illness presents another challenge for the patient and the surgeon, though. There have been reports of recurrence rates as high as 30% in adults and as high as 70% in children. Here, we describe a case of persistent recurrent otorrhea following revision surgery, along with acquired recurrent cholesteatoma following canal wall down surgery. A 38-year -male with underlying Diabetes Mellitus and Hypertension presented with left scanty and foul-smelling ear discharge for 2 years and left reduced hearing. He was diagnosed with left chronic active otitis media with cholesteatoma for which he underwent left modified radical mastoidectomy, meatoplasty and tympanoplasty in 2017. Five months post operatively, he presented with left otorrhea. However, he defaulted followed up and presented in April 2018 for similar complaints. Otoscopy examination revealed left tympanic membrane perforation at poster superior quadrant of pars tensa and bluish discoloration behind pars flacida. He was diagnosed as recurrent left cholesteatoma and subsequently he underwent left mastoid exploration under general anesthesia in June 2018. Postsurgery, he developed recurrent ear discharge which was treated with topical antibiotics and ear toileting. We report a case of recurrent Cholesteatoma despite canal wall down procedure requiring a second redo procedure and with persistent recurrent otorrhea after the redo procedure.However, this case demonstrates the need for regular follow ups even after a canal wall down procedure for detecting recurrence of disease. Moreover, this case denotes some of the patient factors and surgeon factors involved in disease recurrence. Furthermore, importance of opting for an imaging study in case of high suspicion of the disease.

Surgical Option for External Auditory Canal Cholesteatoma: A Case Report.

External Auditory Canal Cholesteatomas (EACC), is an exceptionally rare condition with a prevalence of only 0.1-0.5% among new patients1. EACC are known to possess bone eroding properties, causing a variety of complications, similar to the better-known attic cholesteatomas. We describe here the novel surgical management of a case of EACC. She is 38-year-old female who presented with otorrhea for 6 months. Clinical examination and radiological investigations suggested the diagnosis of an external auditory canal cholesteatoma. The patient underwent modified radical mastoidectomy with type 1 tympanoplasty with meatoplasty. Post-operatively, the patient showed marked clinical improvement.

Finding the Silver Bullet for Persistent Foramen Hushke.

We report a case of persistent foramen Hushke and embark on a literature search from 1990 to 2021. The search was done using electronic databases of PubMed and Google Scholar using the MESH words 'TMJ herniation', 'persistent foramen Hushke', 'TMJ prolapse', 'EAC dehiscence', and 'ear canal mass'. A total of 37 other case reports were included after excluding duplicates and non-English publications. The most common presentations, treatment modalities, complications, and outcomes were discussed. Common presentations include otalgia, tinnitus, otorrhea, and aural fullness. Surgical intervention shows a good outcome, whereas conservative treatment shows a mixed response. Despite the overwhelming success of surgical interventions in treating symptomatic persistent foramen of Hushke, it is still early to establish a guideline to manage these patients, as this condition is rare and presents with variable symptoms. More high-quality studies and a long-term follow-up of the patients may be essential to observe and compare the outcome and recurrence rate of temporomandibular joint (TMJ) herniation.

Exploring Skull Base Osteomyelitis: Comprehensive Case Reports and Management Strategies.

Skull base osteomyelitis is a not commonly encountered but potentially fatal consequence of untreated necrotizing otitis externa. Early recognition and appropriate treatment are crucial to prevent serious complications such as cranial nerve palsies, meningitis, and intracranial abscess formation. The case reports presented in this study provide a rich depiction of the clinical presentation, diagnostic challenges, and interventions employed. Early recognition and appropriate management of skull base osteomyelitis are crucial to prevent complications and improve patient outcomes.

Extensive Base of Skull Osteomyelitis: Why Does It Still Occur?

Skull base osteomyelitis (SBO) is a rare yet serious intratemporal infection that often masquerades as a skull base malignancy. It is most common in diabetic and immunocompromised patients. We present a case of an elderly diabetic patient with end-stage renal disease with progressive malignant otitis externa. The disease progressed to involve the base of the skull, causing multiple cranial neuropathies. Early initiation of intravenous (IV) antibiotics, along with supportive treatment, may improve the long-term prognosis of the disease. This case highlights the importance of keeping a high index of diagnostic suspicion for SBO in patients with risk factors. Early diagnosis and prompt treatment can drastically decrease morbidity and mortality due to SBO.

Prevalence of Hearing Loss among Very Low Birth Weight (VLBW) Babies in Two Tertiary Hospitals in Malaysia.

OBJECTIVE: This study aimed to evaluate hearing loss among very low birth weight babies in two hospitals in Malaysia. MATERIAL AND METHODS: A total of 380 babies from Hospital Canselor Tuanku Muhriz (HCTM), Kuala Lumpur and Sarawak General Hospital (SGH) were recruited in this retrospective study. All babies with birthweight less than 1500grams nursed in the Neonatal Intensive Care Unit (NICU) between January 2014 till December 2019 was included in the study. Data was analysed on demography, interval taken for hearing intervention and defaulter rate. The data of patient parameters between both hospitals were analysed and association between various factors were evaluated. RESULTS: A total 187 Very Low Birth Weight (VLBW) Kuala Lumpur babies and 193 VLBW Sarawak babies met the inclusion and exclusion criteria, among which 10.1% and 10.9% had SNHL in Kuala Lumpur and Sarawak respectively. CHL was reported among 8.6% Kuala Lumpur and 14% of Sarawak babies. When studied on the different types and degrees of hearing loss, 2.6% of Kuala Lumpur babies born less than 28 Weeks Gestation Age (WGA) had moderate SNHL and 2.0% of Sarawak babies had profound SNHL. In this study only gestational age (week) (p=0.003) and dysmorphism (p<0.001) were statistically significant to be associated with hearing loss. CONCLUSION: The prevalence of hearing loss among VLBW babies in Kuala Lumpur was 20.3% and 24.8% in Sarawak. Gestational age (p=0.044) and presence of dysmorphism (p<0.001) were found to have statistically significant association with prevalence of hearing loss. The defaulter rate at Kuala Lumpur was 52.6% and 42.3% in Sarawak.

Factors Affecting Early and Late Cochlear Implantation.

OBJECTIVES: This study aimed to describe the factors affecting early and late cochlear implantation. MATERIALS AND METHODS: A total of 159 patients from the Hospital Canselor Tuanku Muhriz (HCTM) Cochlear Implant Programme were recruited in this retrospective cross-sectional study. All paediatric Cochlear Implant (CI) recipients with pre-lingual deafness were included in this retrospective study. The study was conducted from January 2019 until December 2020. The pre-lingual cochlear implant recipients' data were analysed based on demographics and interval from diagnosis to hearing aid fitting and implantation. The association between the dependent variables with early and late cochlear implantation was compared. RESULTS: A total of 83 (52%) patients were female. Chinese race constituted most of the patients, which was 90/159 (57%). The majority were from middle-income families (M40); 89 (56%). The most common aetiology of Hearing Loss (HL) was idiopathic; 139 (87%), followed by intrauterine infections, which comprised of congenital CMV; 8 (5%) and congenital Rubella; 1 (1%) and nonspecific intrauterine infection 2 (1%). The relationship between the universal neonatal hearing screening and the interval between diagnosis to implantation was significant (p=0.033). Other variables were not significant. CONCLUSION: UNHS was a significant factor contributing to early and late implantation. The median age of diagnosis of hearing loss was 18 months (interquartile range; 15); the age of CI was 34 months (interquartile range; 24); the interval from diagnosis to hearing aid was 2 months (interquartile range; 5), and the interval from diagnosis to CI was 16 months (interquartile range; 14).

Reliability and Accuracy of Chirp Based Multiple Auditory Steady State Response (MSSR) and Auditory Brainstem Response (ABR) in Children.

OBJECTIVE: The purpose of this study was to compare the reliability and accuracy of chirp-based Multiple Auditory Steady State Response (MSSR) and Auditory Brainstem Response (ABR) in children. METHODS: The prospective clinical study was conducted at Selayang Hospital (SH) and Hospital Canselor Tuanku Muhriz (HCTM) within one year. A total of 38 children ranging from 3 to 18 years old underwent hearing evaluation using ABR tests and MSSR under sedation. The duration of both tests were then compared. RESULTS: The estimated hearing threshold of frequency specific chirp MSSR showed good correlation with ABR especially in higher frequencies such as 2000 Hz and 4000Hz with the value of cronbach alpha of 0.890, 0.933, 0.970 and 0.969 on 500Hz, 1000Hz, 2000Hz and 4000Hz. The sensitivity of MSSR is 0.786, 0.75, 0.957 and 0.889 and specificity is 0.85, 0.882, 0.979 and 0.966 over 500Hz, 1000Hz, 2000Hz and 4000Hz. The duration of MSSR tests were shorter than ABR tests in normal hearing children with an average of 35.3 minutes for MSSR tests and 46.4 minutes for ABR tests. This can also be seen in children with hearing loss where the average duration for MSSR tests is 40.0 minutes and 52.0 minutes for ABR tests. CONCLUSION: MSSR showed good correlation and reliability in comparison with ABR especially on higher frequencies. Hence, MSSR is a good clinical test to diagnose children with hearing loss.

Is a Higher Body Mass Index a Risk Factor for Developing Antiphospholipid Antibody Syndrome?

BACKGROUND:  Antiphospholipid antibodies (aPLs) are antibodies directed against components of the cell membrane and can be associated with clinical features or be asymptomatic in 1-5% of the population. OBJECTIVE: The objective of this study is to investigate the frequency of aPL positivity based on body mass index (BMI). METHODS:  This is a retrospective analysis of all aPL testing done in a tertiary center between 2010 and 2020. The difference between patients with BMI <25, BMI 25-30, and BMI>30 is calculated using chi-square or Fisher's exact test as appropriate for categorical variables and a two-sample t-test for numerical variables. Unadjusted then multivariable logistic regression models were conducted to evaluate the effect of BMI on aPL positivity adjusting for age, thrombosis history, pregnancy complications history, and presence of autoimmune disease. Sex was included as an effect modifier. RESULTS:  Among 312 patients, the outcome (any positive aPL) was detected in 26 (20.8%), 13 (13.0%), and 16 (18.4%) patients with BMI groups: BMI <25, BMI 25-30, and BMI > 30, respectively. A multivariable logistic regression showed that those with BMI 25-30 had a lower risk of aPL positivity when compared to patients with BMI <25 (OR of 0.55 CI 0.25 - 1.14, p=0.116), and patients with BMI >30 also carried a lower risk compared with patients with BMI<25 (OR of 0.76, 95% CI 0.36 - 1.56, p=0.46); these results were not statistically significant. INTERPRETATION:  The results suggest that a higher BMI was not a risk factor for aPL positivity. A better understanding of the complex interactions between antiphospholipid antibodies and obesity should be further investigated.

A Rare Case of Idiopathic Bilateral Medial Canal Fibrosis.

Medial canal fibrosis usually occurs as sequelae of known conditions such as trauma, infection, or surgery. Rarely, it occurs without an identifiable cause, hence the term idiopathic medial canal fibrosis. Regardless of the etiology, the reportedly most successful treatment is surgery. A 52-year-old lady presented to us with bilateral reduced hearing and left ear tinnitus. There was no significant history to suggest the possible cause of the symptoms. Clinically, there is the presence of thick solid fibrous in the bilateral ear canal. Audiological examination revealed a conductive hearing loss bilaterally with tympanometry of type B, and imaging was done. She successfully underwent canalplasty following that and is doing well to date. In this paper, we reported a rare case of bilateral idiopathic medical canal fibrosis, and we discuss the causes, diagnosis, and best treatment for this disease.

Malay Version of Nijmegen Cochlear Implant Questionnaire and Quality of Life of Patients with Post-lingual Deafness.

Objective: The Nijmegen Cochlear Implant questionnaire (NCIQ) was used to gauge the quality of life (QOL) improvement among cochlear implant (CI) users who suffered from post-lingual deafness. This study aimed to determine the consistency and reliability of the Malay version of the Nijmegen Cochlear Implant questionnaire (NCIQ-M) and to report the QOL of patients using NCIQ-M. Methods: This study has two phases: Phase I involves the translation of the NCIQ from English to Malay, followed by internal consistency and test-retest reliability assessment of the final version of NCIQ-M. Phase II involves QOL assessment of post-lingual deafness using NCIQ-M. Results: Twenty CI users and 20 non-CI users answered the NCIQ-M. Test-retest reliability analysis of the NCIQ-M was performed using an intraclass correlation coefficient, achieving scores of more than 0.85. Internal consistency was analysed with Cronbach α of more than 0.70 in all subdomains. Scores between the two groups of subjects were analyzed using an independent sample t-test. Good internal consistency, intraclass correlation, and test-retest reliability were obtained. Scores in all six subdomains of the NCIQ-M are significantly higher in the CI user group than in the non-CI user group. Conclusions: The NCIQ-M is a consistent and reliable subjective questionnaire to determine the QOL of CI users concerning physical, psychological, and social functioning.

Acute Facial Nerve Palsy in Facial Nerve Schwannoma Following COVID-19 Infection.

Lower motor neuron facial nerve palsy (FNP) has many causes. Bell's palsy is by far the commonest cause. Among other causes include infective and neoplastic causes. While FNP caused by facial nerve schwannoma (FNS); a benign neoplastic condition of the facial nerve is slowly progressing, infective causes mainly viral origins present with acute FNP. We present a young female who complained of an acute onset of FNP on day five of her COVID-19 infection. She initially presented with symptoms suggestive of ear infection, and subsequent magnetic resonance imaging (MRI) showed evidence of FNS, which she was subjected to surgery later at our center. This rare acute incidence of FNP in schwannoma might be triggered by the COVID-19 infection and demonstrates the role of imaging in finding the cause of FNP.

Early onset of capsular contracture after breast augmentation with implant: report of two cases & review of literature.

Capsular contracture (CC) is a local complication caused by an inflammatory reaction that leads to fibrosis. CC commonly occurs after one year of surgery. Hence, there has been no previously reported early onset of CC. Therefore, we report two unusual cases of early onset of post-breast reconstruction capsular contracture.

Preoperative Challenges in Managing Intraparotid Schwannoma.

Schwannomas are a benign and rare entity that originates from Schwann cells. The majority of schwannomas are found in the head and neck regions and usually involve the intratemporal course of the facial nerve (FN). Isolated extratemporal intraparotid involvement is very rare. It is very challenging to diagnose intraparotid facial nerve schwannoma (PFNS) based on fine needle aspiration cytology (FNAC) preoperatively. We report a case of an intraparotid facial nerve schwannoma masquerading as pleomorphic adenoma. The diagnostic challenges and imaging features along with its management are discussed.

An Unusual Delayed Complication of Rib Graft Microtia Reconstruction After Two Decades: A Case Report.

Microtia comprises a spectrum of congenital malformation characterized by partial deformities of the auricular components to complete absence of pinna. Surgical reconstructions of this anomaly are often complex and technically challenging. Infection, hematoma, and skin necrosis with resultant exposed cartilage graft tend to occur in the early phase of post-operative care. Herein, we report a case of a spontaneous auricular abscess with exposed cartilage framework 20 years following rib cartilage reconstruction. To our knowledge, this is the first case of such an occurrence. The treatment options are discussed, along with the review of the literature.

Ceruminous Adenoma: A Rare Mass that Impair Hearing.

Ceruminous adenoma is described as a glandular neoplasm of ceruminous glands. It is seen for less than one percent of all external ear tumours. Ceruminous adenoma cases were reported to have recurrence and residual tumour, however there was no malignant transformation known in ceruminous adenoma up to this date. Here, we report a young adult woman with two years history of progressive reduced hearing and tinnitus of the right ear. She was proven to have right moderate conductive hearing loss with pure tone audiometry. There was a cystic mass with serous content arising from the posterior wall of the right ear canal. We proceeded with wide local excision of the mass via trans-canal approach. Ceruminous adenoma was confirmed with histopathology and immunohistochemistry of CK7 staining. The hearing impairment was resolved completely post excision and there was no recurrence of the tumour on one year follow-up. We concluded wide local excision with appropriate margin of the mass is adequate to prevent recurrence in ceruminous adenoma cases.

Mastoid Abscess in a Child With Eustachian Tube Dermoid Cyst.

A dermoid cyst (DC) is a benign tumor caused by inclusion errors during embryogenesis. DC of the head and neck is a well-recognized entity both clinically and histologically; however, it rarely occurs in the Eustachian tube (ET). Due to its anatomical position, significant morbidity related to middle ear dysfunction may result from ET obstruction. In this report, we present a rare case of a girl child aged two years and nine months with persistent otorrhea, who was initially diagnosed with acute otitis media with mastoiditis, along with suspicion of congenital cholesteatoma. However, high-resolution CT (HRCT) temporal and MRI of the neck revealed a DC of the ET causing left chronic otitis media (COM) with mastoid abscess. The patient underwent mastoid exploration surgery and myringotomy with grommet insertion. Although complete excision is the standard treatment modality for DC, the treatment of poorly ventilated mastoid and middle ear takes precedence over it. MRI surveillance scan is recommended in such cases.