Congenital oval window aplasia: An unusual cause of conductive hearing loss in an adult

@#<p style="text-align: justify;"><strong>OBJECTIVES:</strong> To  report  a  case  of  congenital  oval  window  aplasia  (COWA)  in  a  Filipino  adult presenting   with      unilateral   maximal   conductive   hearing   loss   and   discuss   the   diagnostic considerations, pathophysiology and management.<br /><strong>METHODS:</strong><br /><strong>Design:</strong> Case Report<br /><strong>Setting:</strong> Tertiary Public Referral Center<br /><strong>Patient:</strong> One <br /><strong>RESULTS:</strong> Audiometric evaluation showed a maximal unilateral left conductive hearing loss. High resolution temporal bone CT showed absence of the oval window on the left along with facial and stapes  abnormalities.    Exploratory  tympanotomy  showed  an  aberrant  facial  nerve,  monopodal and  abnormally  located  stapes  and  absent  oval  window. Postoperative  hearing  gain  achieved after a neo-oval window and Schuknecht piston wire prosthesis remained stable over two years.<br /><strong>CONCLUSION:</strong> A congenital minor ear anomaly classified as Cremers Class 4a in which a congenital oval  window  aplasia  was  associated  with  an  aberrant  facial  nerve  anomaly  and  a  monopodal stapes is reported. Recent literature supported the view that congenital oval window aplasia can in selected cases be amenable to various surgical approaches and a stable postoperative hearing gain is achievable in the long term.</p>

Cost-analysis of universal newborn hearing screening in the Philippines

OBJECTIVE: The prevalence of congenital bilateral permanent profound hearing loss in the Philippines is 1.3 per 1000 live births. The prevalence increases to 22 per 1000 live births for unilateral mild to moderate hearing loss. This study was conducted to determine the cost of establishing a universal newborn hearing screening (UNHS) program. Local prevalence data and current costs of screening, diagnostics and intervention strategies for bilateral permanent hearing loss were utilized to estimate the costs of implementing the program. METHODS: Both short-term and long-term costs for hearing screening centers and for families caring for hearing-impaired children were determined using a societal perspective. Calculations included cost of hearing loss and the effectiveness of testing strategies. In this study the societal cost was considered although some of the costs pertained to costs borne by individual patients or their families since none of the screening, diagnostic and intervention strategies are paid for by insurance companies. An exception is the partial subsidy for cochlear implantation that is reimbursable with the Philippine Health Insurance Corporation. RESULTS: Using published data on the prevalence of hearing loss and experience from a pilot universal newborn hearing screening project at a national tertiary hospital (Philippine General Hospital), the long-term benefits and savings from UNHS on a national scale greatly outweigh the immediate costs of testing and intervention. CONCLUSION: The cost benefit of UNHS program at a national level outweighs the financial burden of hearing impaired individuals and their families.

When do aided auditory thresholds reach the speech spectrum after cochlear implant switch on?

OBJECTIVES: This paper aims to determine the length of time before a cochlear implantee would reach aided hearing threshold of METHODS: From January 2009 to February 2011, the aided earing thresholds of 41 prelingually deaf children with cochlear implants after switch on were reviewed. RESULTS: There were 21 males and 20 females. Majority of children 7 years old reached it at CONCLUSION: Sixty-six percent of the children with cochlear nplants reached the target threshold within the first 3 months post fitting and 80% within the first 6 months post fitting. More of the older children reached target threshold earlier than the younger children. There was a trend that more females reached the target threshold earlier than the males but this was not statistically significant.

Cochlear implantation in chronic otitis media

OBJECTIVES: The objectives of this study are to describe the experience on cochlear implantation among patients with chronic otitis media (COM) and to describe the management of patients with otitis media before and after cochlear implantation (CI). METHODS: The records of one hundred five consecutive patients who have undergone cochlear implantation were reviewed and the patients with chronic otitis media either before or after surgery were included in the study. Data such as age at implantation, etiology of deafness, laterality of otitis media, radiologic studies, medications and intraoperative findings in these patients were reviewed then recorded in a separate database file. Outcomes in terms of complications during or after surgery were likewise noted. RESULTS: Of 105 patients who had cochlear implantation, twelve patients had history of chronic otitis media or recurrent acute otitis media. There were three patients, 2 adults with bilateral COM as the cause for deafness and one child with bilateral COM and middle ear foreign body mimicking a cholesteatoma. All three had to undergo mastoidectomy and tympanoplasty for the surgical management of active suppurative chronic otitis media. No further episodes of otitis media were noted and all were otitis free subsequently at the time of cochlear implantation. Five prelingually deaf children had otitis media following cochlear implantation (one week to 4 months), two of whom had to undergo revision cochlear implant surgery without explantation for definitive management. Four additional patients had episodes of recurrent otitis media prior to and after cochlear implantation which required only medical management with broad spectrum.antibiotics. CONCLUSION: Despite high prevalence of otitis media in the Philippines, our data supports literature reports that cochlear implantation is a safe and effective procedure in patients with bilateral profound hearing loss both in the pediatric and adult.

Auditory brainstem response latencies of infants and maternal eposure to environmental toxic product

OBJECTIVE: To explore the effects of pesticide exposure on the auditory system, specifically on hearing status based on auditory brainstem responses. METHODS: A cohort of pregnant women was identified in several communities in a rural area from April 2002 to February 2003 and followed up until delivery. Mother-infant dyads were assessed for exposure to pesticides. Maternal and fetal exposures to environmental toxic products were determined by measuring levels in maternal hair and blood, and infant cord blood, hair, and meconium, respectively. Hearing status was measured using otoacoustic emissions (OAE) and confirmed by diagnostic auditory brainstem responses (ABR) measured at 80, 60, and 40 decibels. Waves I, III, V were identified and absolute latencies measured, including inter-peak latencies from waves 11III, I-V, and III-V. Pesticide exposure was then correlated with latencies of Waves I, III, V, and interpeak latencies of waves I-III, IIV, and III-V. Hearing loss and pesticide exposures were correlated with Griffiths Mental Development Scores (GMDS). RESULTS: Significant delays in the ABR wave latencies were noted in the group with exposure to pesticides. Propoxur was the most common toxic product detected in infants and meconium the best substrate for its detection. There was a 1.4% risk of hearing loss with exposure to propoxur (RR=0.52 (0.12-2.30), p = 0.06), a 6.25% risk with cypermethrin exposure (RR= 4.53 (0.61133.64), P = 0.10) and 6.25% risk with pretilachlor exposure (3.13 (0.44-22.30), p = 0.07). Griffith's Mental Developmental Scale scores (GMDS- hearing and speech subscale and general quotient scores) were not significantly different between exposed and unexposed groups. However, three infants with positive exposures and hearing loss had below average, or low to average scores using this scale. CONCLUSION: Maternal exposure to environmental toxic products may affect the auditory pathway in infants at birth. Pregnant women should limit their exposure to such toxic products in order to avoid neurodevelopmental effects particularly on hearing because this is very important in the critical stage of language and speech development.

Training of nurses in ear examination and hearing screening in the school setting (Phase II): A cross-sectional study

OBJECTIVES:To determine the agreement between 1) ear examination findings of the otorhinolaryngologist using an otoscope and trained elementary school nurses using a penlight, 2) hearing screening findings of the otorhinolaryngologist and elementary school nurses, both using the Philippine National Ear Institute (PNEI) Method of 512 Hz Tuning Fork Test (TFT) and 3) PNEI Method of 512 Hz TFT findings and screening audiometry findings.METHODS:In this continuing study, nurses residing in the venue of the study, and previously trained in ear examination using a penlight and hearing screening using the PNEI 512 Hz TFT, conducted these in school children who attended the hearing screening and medical mission. Otoscopy, PNEI Method of 512 Hz TFT, and screening audiometry were then conducted on the children by the otolaryngologist. The nurses and the otolaryngologist performed independent and blinded assessments.RESULTS:Eighteen nurses and ninety children participated in the study. Data subjected to Kappa statistics showed good agreement between nurses and otorhinolaryngologist's findings in the examination of the external canal and tympanic membrane and in PNEI Method of 512 Hz TFTs, and between the PNEI Method of 512 Hz TFT and screening audiometry.CONCLUSION:PNEI methods of penlight ear examination and 512 Hz TFT may be effective tools for early detection of common ear conditions and hearing screening in Filipino school children. These may be conducted in the school setting not only by otorhinolaryngologists but also by adequately trained school nurses.

Temporal bone paraganglioma

OBJECTIVE: The aim of the study was to describe the diagnosissurgical management and outcomes of eight patients with bone paraganglioma.PATIENTS: A series of 8 patients with temporal bone paraganglioma were seen over a 10-year period and operated n by the senior author.RESULTS: The majority of patients presented with hearing loss and pulsatile tinnitus. Other symptoms were facial numbness, facial asymmetry, dizziness, hoarseness, and dysphagia. All patients had pre-operative high resolution computed temography (HRCT) scan and/or magnetic resonance imaging. Pre-operative angiography and embolization facilitated the excision of these tumors in all but one case. There was reversal of pre-existent cranial nerve neuropathy in 3 of 8 cases. Unique to this series were the management of a patient who previously underwent gamma knife radiosurgery, one case in whom the glomus tumor grew from the better hearing ear thus posing the surgical management challenges and a complicated case of postoperative necrosis that required latissimus dorsi flap reconstruction. Other complications were described including post-operative facial paresis which eventually resolved in all patients.CONCLUSION: In our setting, it is possible to achieve good result with acceptable morbidity with surgery with or without postoperative radiotherapy.

Training of nurses in ear examination and hearing screening in the school setting.

The objectives of this study were to determine the agreement between the ear examination findings of the otorhinolaryngologist (trainer) and the elementary school nurse (trainee) after training with the use of a penlight and to determine the mean sound pressure level (SPL) produced by school nurses as a standard parameter for hearing screening using a 512 tuning fork after training on tuning fork testing by the otorhinolaryngologist. Training workshops in ear examination using a penlight and hearing screening using a 512 tuning fork were conducted for school nurses. Data for assessment of ear examination skills and production of SPL were collected by questionnaire and observation of performance. Kappa statistics were used to assess agreement between trainees' and trainer's responses. Mean and standard deviation were determined for the assessment of the SPL produced. Results showed an excellent agreement between the school nurses' and otorhinolaryngologist's observations on ear examination. These included observations of the ear canal, visualization of the tympanic membrane and identification of unusual findings such as wax and discharge. The majority of nurses responded positively in terms of the ease and confidence in performance of the procedure. Regarding tuning fork testing, the nurses were able to produce significant SPL. The mean SPL produced by the nurses using a 512 tuning fork was 56.316 dB.