Pediatric hearing loss: common causes, diagnosis and therapeutic approach.

Pediatric hearing loss limits the child ability to develop effective auditory and speech capabilities. Early rehabilitation of hearing loss results in higher levels of linguistic, academic and social skills. To achieve the best outcomes, proper and timely diagnosis is essential. The etiology of hearing loss can be classified according to its nature as sensorineural hearing loss (SNHL), conductive hearing loss (CHL) or mixed hearing loss (MHL). Congenital hearing loss could be genetic or non genetic (acquired), syndromic or non-syndromic. Hearing loss can be classified according to the severity into mild (26-40 dB HL), moderate (41-70 dB HL), severe (71-90 dB HL) and profound (more than 90 dB HL). Management of hearing loss is mainly influenced by the nature, the bilaterality, the severity and the age at diagnosis. Severe to profound bilateral SNHL can be managed by cochlear implantation (unilateral or bilateral), if picked up at early age while mild to moderate bilateral SNHL are easier to manage with conventional hearing aids. CHL has less impact on the speech development of the child. It is usually managed by correcting the underlying etiology such as otitis media with effusion or even surgically in cases of external ear atresia or ossicular malformations. Unilateral SNHL have fortunately no impact on the language development of the child and can be passed undiagnosed until preschool-aged children. The implementation of national newborn hearing screening programs has improved the management of affected children by rehabilitating them at early stages to allow for normal speech development. In this review article, we aim to highlight the most common causes of pediatric hearing loss, their character and presentation and to review the diagnostic and therapeutic approaches of a deaf child.

Intratympanic steroid injection as a salvage treatment for sudden sensorineural hearing loss.

OBJECTIVE: To determine the efficacy of intratympanic methylprednisolone injections for treating sudden sensorineural hearing loss. METHOD: A retrospective chart review was performed to identify patients suffering from sudden sensorineural hearing loss with no recovery after oral steroids. Patients were given up to three intratympanic methylprednisolone injections at one-week intervals. They were classified according to their functional hearing class, remission was monitored and potential factors affecting prognosis were analysed. RESULTS: Intratympanic injections provide effective salvage therapy for sudden sensorineural hearing loss (p = 0.039). Changes in pure tone average and speech discrimination score were analysed following intratympanic methylprednisolone injections. The pure tone average reached a plateau after the second injection; however, the speech discrimination score improved until after the third injection. Hearing improvement after intratympanic injections mainly occurred at low frequencies. The interval between symptoms appearing and intratympanic injections starting was not significantly associated with remission (p = 0.680). CONCLUSION: A delay between symptom onset and the first intratympanic methylprednisolone injection does not seem to affect prognosis.

Canal wall window mastoidectomy for extensive labyrinthine cholesteatoma: total dissection and hearing preservation.

This case report highlights outcomes of a 6-year-old patient who preserved functional hearing after complete dissection of an extensive labyrinthine cholesteatoma causing two semicircular canals fistulas with endolymph leak, tympanic and labyrinthine fallopian canal erosion of the facial nerve and internal auditory canal invasion with cerebrospinal fluid leak. The patient preserved 40 dB average of bone conduction threshold and 92% of speech discrimination score at 26 months postoperatively. This article reveals that canal wall window mastoidectomy might be an option even in cases of extensive cholesteatomatous labyrinthine fistula therefore avoiding hearing loss and long life cleaning of a canal wall down mastoid cavity.

Prognostic indicators for sensorineural hearing loss in temporal bone histiocytosis.

OBJECTIVE: Our review aims: (1) to highlight the suspicion of sensorineural hearing loss (SNHL) in temporal bone Langerhans' cell histiocytosis (LCH); (2) to assess the evolution of SNHL in this pathology; (3) to identify radiologic findings of the otic capsule invasion by LCH; (4) to determine prognostic factors for SNHL in temporal bone LCH. METHODS: We performed a literature review through MEDLINE for SNHL in temporal bone LCH related articles that were published between 1954 and 2008. We identified 12 related studies of which 18 patients were noted. We also added our case to this series. The information from the reports was analyzed to characterize the clinical and demographic data and to focus on the sensorineural hearing aspect of the disease and on the damage of the semicircular canals (SCC) and the cochlea. RESULTS: Ten percent of patients with temporal bone LCH presented SNHL. The mean age of patients is 3.5 years among children and 35.5 years among adults. Male to female ratio is 1:1.14. There were 13 unilateral cases, 6 bilateral cases and 1 case of multisystemic histiocytosis. Cochlea and SSCs were involved in 4 and 20 temporal bones, respectively. The lateral SCC is the most frequently eroded canal. In 23 ears hearing level was reported: 15 ears had a SNHL and the remaining was a mixed or a conductive hearing loss type. 10 ears suffered from a profound hearing loss and none of them improved after treatment regardless otic capsule affected structures. In all cases of normal hearing, moderate hearing loss and severe hearing loss before treatment cochlea have not been affected. However a single or two semicircular canals invasions have been noted. Where it is reported (15 out of 25 temporal bone) there was a radiological healing of the otic capsule lesion after treatment. Remineralization occurs 6 months after treatment. CONCLUSION: In LCH, hearing loss level before treatment can be considered as a prognostic indicator for hearing in response to treatment. Even though bone mineralization is essential for a hearing improvement, radiological healing is not a prognostic factor for better hearing level after treatment.

Trigeminal nerve haemangioma eroding the petrous carotid canal.

OBJECTIVE: To report the first case of mandibular branch haemangioma of the trigeminal nerve causing erosion of the petrous carotid canal. The radiological and histological findings in this case are reviewed. CASE REPORT: A 60-year-old woman presented with severe, right-sided facial pain and paraesthesia. There were no associated symptoms of facial weakness or diplopia. A magnetic resonance imaging scan with gadolinium enhancement was performed. This showed a lesion slightly compressing the right Meckel's cave and eroding the right petrous carotid canal, occupying the foramen ovale and extending to the pterygoid muscle. The lesion was removed via a subtemporal approach. CONCLUSION: Haemangiomas are usually found on the skin and in other soft tissues. However, this rare tumour should also be considered in the differential diagnosis of lesions occupying Meckel's cave and the foramen ovale.

Langerhans' cell histiocytosis of the temporal bone in children.

OBJECTIVE: Langerhans' cell histiocytosis (LCH) is a rare pathology that implies an abnormal proliferation of these kinds of cells associated with a granular infiltration that affects different structures of the human body, including the temporal bone. The authors present their series of LCH of the temporal bone in children at the Sainte-Justine university hospital. The twofold objective of this study is to illustrate the clinical presentation, management and prognosis of this disease, and to compare these results with previously reported series. METHODS: A retrospective study was conducted between 1984 and 2007 with patients diagnosed and treated for a LCH of the temporal bone at the Sainte-Justine university hospital, a paediatric tertiary care center. A chart review was performed to obtain demographic, clinical, paraclinical, and therapeutic data. They were analysed and compared to other published series. Through a MEDLINE query, we found that since 1966, 50 articles dealing with a LCH of the temporal bone have been published. RESULTS: Fifty-nine cases of LCH were diagnosed and among them, 10 children had temporal bone involvement. They were four females and six males with a mean age of 3.28 years. The two most frequent clinical manifestations were the presence of a mass in the temporal region (70%) and otitis (60%). Two of our patients had a unifocal lesion of the temporal bone implicated. Eight patients had a multisystem involvement among which, two showed evidence of organ dysfunction. In 80% of cases, the diagnosis was made by immunohistochemical findings of the S-100 protein and/or the CD1 antigen. The common radiological finding on a skull CT scan is a lytic lesion in the temporal bone. Seven patients were treated by chemotherapy, two were treated by radiotherapy as a primary treatment, and one received radiotherapy for a recurrence on the pituitary gland. Finally, one patient was treated with local steroid injections. Two patients had a recurrence. All our patients were in total remission on a mean average of 1.6 years after the diagnosis. Our results concord with other studies in which the prognosis of unifocal bone disease is excellent and children with a multifocal disease have a survival rate of 65-100%. CONCLUSION: LCH is a rare disease. A high-index suspicion should be raised in the context of a temporal mass, chronic otitis, and otorrhea. A biopsy is recommended in the presence of a temporal bone lytic lesion. Chemotherapy is the preferred therapeutic modality.

Temporal bone rhabdomyosarcoma in children.

OBJECTIVE: Rhabdomyosarcoma is the most frequent soft tissue sarcoma in the pediatric age group. The authors present their series of rhabdomyosarcoma of the temporal bone in children at Saint Justine Hospital. The twofold objective of this study is to illustrate the clinical presentation, management, and prognosis of this malignant striated muscle tumor, and to compare these results with previously reported series. METHODS: A retrospective study was conducted of patients diagnosed and treated for rhabdomyosarcoma of the head and neck at Saint Justine Hospital, a tertiary pediatric center, between 1970 and 2005. Only cases of temporal bone rhabdomyosarcoma were included in the study. A thorough review of medical and surgical charts was performed to obtain demographic, clinical, paraclinical, and therapeutic data, which were subsequently analyzed and compared to published results. A MEDLINE search yielded 34 studies dealing with temporal bone rhabdomyosarcoma since the year 1966. RESULTS: Thirty-nine patients with rhabdomyosarcoma of the head and neck region were identified, among which only six children had temporal bone rhabdomyosarcoma. The mean age at the time of diagnosis was 4.15 years. Chronic otitis media was the most common clinical presentation. Five children had the embryonal subtype and one had the botryoid subtype on histology. All patients except two received combined chemotherapy and radiotherapy as treatment. Five-year survival rate was 66%. Our results match those reported in the literature 41-81%. CONCLUSION: Rhabdomyosarcoma of the temporal bone is an aggressive tumor that clinically simulates chronic otitis media. A high index of suspicion should be raised in the context of otitis media that is unresponsive to ordinary medical treatment. A biopsy is hence recommended in the presence of polyps in the external auditory canal that are resistant to medical treatment. Early diagnosis and the adoption of multimodal therapy offer the best outcome.

Hearing preservation mastoidectomy in otogenic meningitis secondary to an enlarged vestibular aqueduct. Case report.

In pyogenic meningitis resulting from a life-threatening ear infection, mastoidectomy is performed as part of the management of the disorder. A dilemma arises when the active ear is the only hearing ear. An active unsafe ear can lead to sensorineural hearing loss whereas ear surgery carries the risk of inner ear damage. We present the case of a 40-year-old woman admitted for severe purulent meningitis and sub-coma secondary to a left mastoiditis with mixed hearing loss on the left side and complete deafness on the right side. The study of this case shows that the intracranial complication was secondary to an abnormally enlarged left vestibular aqueduct. Because of the failure to control meningitis with medical treatment using highly specific antibiotherapy for two weeks, we proceeded with a left side mastoidectomy and closure of the external aperture of the vestibular aqueduct with a muscle graft. This surgery saved the patient's life, cured the meningitis and brought a recovery of a near normal hearing to the only hearing ear. Although demonstrating a rare etiology of intracranial complication, this case confirms that mastoidectomy, even on the only hearing ear, has to be done as early as possible to remove the source of infection, to prevent further intracranial complication, to arrest the progress of the ear disease and preserve or even recover almost normal hearing.

Pharmacological measures to prevent post-operative urinary retention; a prospective randomized study.

One hundred patients undergoing vaginal surgery for genital prolapse were randomly allocated to one of four post-operative management groups which included a control group and three groups receiving differently acting pharmacological agents (distigmine bromide, phenoxybenzamine hydrochloride, and prostaglandin F2 alpha) variously reported as being useful in preventing urinary retention after vaginal surgery. The incidence of an elevated residual volume in the control group was 10.7%. All the pharmacological agents appeared to increase by about three times the incidence of an elevated residual urinary volume with statistical significance (P less than 0.05) being noted for distigmine bromide and PGF2 alpha. While this increased incidence occurred irrespective of the type of surgery in the case of distigmine bromide and phenoxybenzamine . HCl, the increase was most marked (P less than 0.01) when PGF2 alpha was used after anterior repair surgery.