Mucosal melanocytic lesion in the middle ear extending to the inner ear and nasopharynx.

Benign dendritic melanocytic proliferation is usually observed in the skin. We report an extremely rare case of a melanocytic lesion in the middle ear mucosa. Only 3 cases of melanocytic lesions in the middle ear have been reported. Our report is the only one that describes an adult with a melanocytic lesion in the middle ear that extended to the inner ear and nasopharyngeal mucosa. A 23-year-old female presented with profound sensory neural hearing loss and recurrent discharge from the right ear. Examination revealed a blue right tympanic membrane with a small perforation. Computed tomography scans and magnetic resonance imaging were performed. After surgical exploration and histopathological examination, the patient was found to have a benign melanocytic lesion in the right middle ear. Melanocytic lesions are commonly found in the skin. There are no previous reports have described adult patients with melanocytic lesions in the middle ear that extend to the inner ear and nasopharynx, which cause profound hearing loss with recurrent ear discharge. These patients require regular follow-up to assess the progression of the lesion and to watch for any malignant behavior.

Middle Ear Implant in a Patient With Fibrous Dysplasia: An Alternative for Hearing Restoration.

We propose middle ear implants (MEI) as alternative hearing restoration option for patients with fibrous dysplasia of the temporal bone (FDTB). A 60-year-old man presented with unilateral right-sided conductive hearing loss (CHL) and FDTB. The Vibrant Soundbridge (VSB) MEI was implanted in the right ear. Preoperatively, the right side had a pure tone average (PTA) threshold of 67 dB, speech reception threshold (SRT) = 75 dB HL, and speech discrimination score (SDS) = 54% at 80 dB HL presentation level. Postoperative aided PTA threshold of the right ear was 28 db, aided SRT = 30 db, and SDS = 96% at 65dB HL. After excluding cholesteatoma, VSB provides an alternative hearing restoration technique for adults with FDTB and CHL due to stenosis of the external auditory canal and/or ossicular crowding.

The association between consanguineous marriage and offspring with congenital hearing loss.

BACKGROUND: Consanguinity is a commonly recognized practice among marriages in the Middle East and may lead to an increase in the prevalence of inherited disorders. Autosomal recessive deafness is the most common form of inherited congenital hearing loss (CHL). OBJECTIVES: Determine the association of consanguineous marriages with congenital sensorineural hearing loss (SNHL) and auditory neuropathy. DESIGN: Descriptive and analytical cross-sectional study. SETTING: Ear specialist hospital. PATIENTS AND METHODS: Children with severe-to-profound congenital SNHL, who had been referred to the specialist hospital for cochlear implant were analyzed. Patients were divided into subgroups based on degree of consanguinity. MAIN OUTCOME MEASURE: The relative risk of having more than one child with SNHL in offspring of a consanguineous marriage. SAMPLE SIZE: 189 parents and children with CHL. RESULTS: The parents of 157 children (83.1%) were blood-related. Of those, 48 had more than one child with CHL (31.4%), while only two parents who were not blood-related had more than one child with CHL (6.25%; P=.005). Among the 189 children, 131 (69.3%) parents were direct cousins. Only 39 (20.6%) and 43 (22.8%) children had family histories of CHL on the paternal and maternal sides, respectively. There was no statistically significant difference in the prevalence of auditory neuropathy between the offspring of consanguineous and non-consanguineous marriages (P=.648). CONCLUSION: The risk of having more than one child with SNHL in the offspring from a consanguineous marriage is 3.5 times higher than that of a non-consanguineous mating. LIMITATION: The association of hearing loss degree with consanguinity was not studied. CONFLICT OF INTEREST: None.

Validation and inter-rater reliability testing of the Arabic version of speech intelligibility rating among children with cochlear implant.

OBJECTIVES: To validate and assess the reliability of the new version of an Arabic speech intelligibility rating among di erent raters. METHODS: This cross-sectional analysis was carried out between December 2018 and January 2019. Thirty cochlear-implant (CI) children (study group) and 30 subjects (control group) were enrolled. Study candidates' speech skills were evaluated using the translated Arabic SIR by parents and original SIR by professions such as speech-language pathologists (SLPs). Inter-rater agreement, test-retest reliability, pre- and post-intervention score (responsiveness test), patient versus control score comparison (discriminant validity), and cross-validation of Arabic SIR have all been assessed. RESULTS: There was a good sense of agreement between the post-operative SIR parents' assessments and the professional SLPs' assessments (r=0.920, p less than 0.001). The mean of study subjects pre- and post-implantation score of Arabic SIR showed a statistically significant difference (p less than 0.001). CONCLUSION: The Arabic SIR demonstrated excellent reliability with strong consistency. It showed its clinical ability in distinguishing healthy subjects from patients along with follow up of speech development skills over time. The Arabic SIR can be used by parents to evaluate post-CI progress of their children.

Cochlear electrode array tip fold-over in incomplete partition-I - A case report.

Electrode tip fold-over is a rare complication of cochlear implantation (CI), especially in lateral wall (LW) electrodes. We describe a case of LW electrode tip fold-over in a patient with incomplete partition type-1 (IP-I). Preoperative measurement of cochlear length is important for optimal CI electrode selection. In cochlear malformation, Stenvers x-ray is superior to spread of excitation in detecting tip fold-over. Despite tip fold-over, hearing threshold, speech reception threshold, and word recognition score were almost symmetrical bilaterally. Therefore, revision surgery should be reserved for patients with persistent symptoms or markedly affected speech performance, in whom deactivation did not suffice.

Relationship between proximity to a cochlear implant center and early presentation in children with congenital hearing loss.

OBJECTIVES: To  determine whether distance from a cochlear implant (CI) center can influences the ages of presentation and intervention for CI, which thus may influence CI outcomes in Saudi Arabia. METHODS: This study comprised a retrospective cross-sectional analysis of 221 patients who presented to the CI committee between March 2016 and March 2018 at King Abdullah Ear Specialist Center, Riyadh, Saudi Arabia. Through phone interviews and patients' files, age at suspicion, audiology testing, diagnosis, hearing aid fitting, and CI discussion were recorded for prelingually deaf children; additionally, demographic information were recorded. Patients were divided into 3 groups based on the distance between their place of residence and the nearest CI center (residing within 200 km, 200-500 km, and greater than 500 km). RESULTS: Patients living within 200 km showed the youngest mean age for all tested checkpoints; however, there were no statistically significant differences among the groups. CONCLUSION: In Saudi Arabia, distance from CI center does not have a significant effect on age of presentation for CI intervention.

Inter-rater Reliability and Validity of the Arabic Version of Categories of Auditory Performance-II (CAP-II) Among Children With Cochlear Implant.

BACKGROUND: Categories of Auditory Performance II (CAP-II) is an established audiological test that consists of a rating scale that can be used for prospective assessment of the auditory performance of cochlear implant (CI) recipients. OBJECTIVE: To create and validate an Arabic version of Categories of Auditory Performance scale II (CAP-II), and to evaluate its reliability. METHODS: In this cross-sectional study, 30 post-CI children (patients' group) along with 30 control subjects were included. The translated Arabic CAP-II scale has been used by speech-language pathologists and parents to evaluate the study subjects' auditory abilities. The interrater reliability, test-retest reliability, pre and postintervention score (responsiveness test), case versus control score comparison (discriminate validity), and cross-validation of the new Arabic version of CAP-II have been assessed. RESULTS: The Arabic CAP-II demonstrated high test-retest and inter-rater reliability. There was a positive correlation between the Arabic CAP-II and Meaningful auditory integration scale. The Arabic CAP-II scores were higher in the patients' group compared with control subjects. CONCLUSION: The Arabic CAP-II showed high inter-rater reliability with excellent concurrent and discriminate validity. The Arabic version of CAP-II seems to be a valid and reliable tool in assessing different auditory performance in Arabic speaking children with hearing impairment.

After a first prelingually deaf child, does the family learn a lesson?

BACKGROUND: Congenital sensorineural hearing loss (SNHL) is a common disability in children. It can affect normal language development and educational achievement. Today, the time to cochlear implant is delayed for many children, which in turn delays intervention and impacts outcomes. Lack of knowledge and experience with congenital SNHL in the family are critical factors that can delay identification and intervention. OBJECTIVES: Compare treatment seeking behavior in families for a first and second congenitally deaf child. DESIGN: Analytical, cross-sectional using medical record data. SETTING: Ear specialist hospital in Riyadh. SUBJECTS AND METHODS: All patients who presented to the cochlear implant committee from March 2016 to March 2018 and met criteria were included in the study. Data on when the subjects presented to hospital and were approved for cochlear implant were retrieved from the patient files and through phone calls to the family. The age of first suspicion, audiological testing, diagnosis, hearing aid fitting, and the decision for cochlear implant were compared between the first and second child in families with multiple children with congenital SNHL. MAIN OUTCOME MEASURES: The timing difference between the first and second deaf child in seeking treatment. SAMPLE SIZE: 116 (58 pairs). RESULTS: The second child was suspected to have hearing loss 13.6 months earlier than the first child and presented to the cochlear implant committee for final decision 16.7 months earlier than his\her sibling. Differences in the mean ages at suspicion of hearing loss, presentation to the hospital for audiological evaluation, hearing aid fitting, diagnosis, and decision for cochlear implant by cochlear implant committee were statistically significant ( P<.001). CONCLUSION: Experience and knowledge has a major effect on early identification. We need to implement educational programs for the public to increase awareness of how to recognize a deaf child and what steps to take. LIMITATIONS: Single-centered. CONFLICT OF INTEREST: None.

Familial hypercholesterolemia with bilateral cholesterol granuloma: A case series.

INTRODUCTION: Cholesterol granuloma is a benign mass that commonly involves the petrous apex but rarely affects other structures, such as the mastoid cavity. It is diagnosed histologically by the presence of giant cells, and Its management is individualized based on some factors such as the size and location of the lesion. PRESENTATION OF CASE: The first case was a 33-year-old man who presented to the outpatient clinic with a two-year history of right-sided pulsatile tinnitus, hearing loss, and vertigo. Upon investigations, a large, destructive mass in the tympanomastoid region was found and managed surgically. The other case was for a 41-year-old man who presented to the emergency department with loss of consciousness. Urgent CT was done and revealed an aggressive hypodense posterior fossa mass destroying the right temporal bone that was managed surgically. DISCUSSION: In this report, we describe two patients with familial hypercholesterolemia who developed bilateral cholesterol granuloma that were managed surgically. CONCLUSION: These cases are reported because of their rare location and presentation since few cases of bilateral cholesterol granuloma have been reported in the literature.