Hearing optimisation in neurofibromatosis type 2: A systematic review.

BACKGROUND: It is common for patients with neurofibromatosis type 2 to develop bilateral profound hearing loss hearing loss, and this is one of the main determinants of quality of life in this patient group. OBJECTIVES: The aim of this systematic review was to review the current literature regarding hearing outcomes of treatments for vestibular schwannomas in neurofibromatosis type 2 including conservative and medical management, radiotherapy, hearing preservation surgery and auditory implantation in order to determine the most effective way of preserving or rehabilitating hearing. SEARCH STRATEGY: A MESH search in PubMed using search terms (('Neurofibromatosis 2' [Mesh]) AND 'Neuroma, Acoustic'[Mesh]) AND 'Hearing Loss' [Mesh] was performed. A search using keywords was also performed. Studies with adequate hearing outcome data were included. With the exception of the cochlear implant studies (cohort size was very small), case studies were excluded. EVALUATION METHOD: The GRADE system was used to assess quality of publication. Formal statistical analysis of data was not performed because of very heterogenous data reporting. RESULTS: Conservative management offers the best chance of hearing preservation in stable tumours. The use of bevacizumab probably improves the likelihood of hearing preservation in growing tumours in the short term and is probably more effective than hearing preservation surgery and radiotherapy in preserving hearing. Of the hearing preservation interventions, hearing preservation surgery probably offers better hearing preservation rates than radiotherapy for small tumours but recurrence rates for hearing preservation surgery were high. For patients with profound hearing loss, cochlear implantation provides significantly better auditory outcomes than auditory brainstem implantation. Patients with untreated stable tumours are likely to achieve the best outcomes from cochlear implantation. Those who have had their tumours treated with surgery or radiotherapy do not gain as much benefit from cochlear implantation than those with untreated tumours. CONCLUSIONS: This review summarises the current literature related to hearing preservation/rehabilitation in patients with NF2. Whilst it provides indicative data, the quality of the data was low and should be interpreted with care. It is also important to consider that the management of vestibular schwannomas in NF2 is complex and decision-making is determined by many factors, not just the need to preserve hearing.

A preliminary randomised controlled trial evaluating the efficacy of saline douching following endoscopic sinus surgery.

OBJECTIVE: To assess whether saline reduces postoperative signs following endoscopic sinus surgery (ESS). DESIGN: Within subject, single blinded randomised controlled trial. SETTING: Single secondary referral centre. PARTICIPANTS: Adults undergoing bilateral ESS for chronic rhinosinusitis or nasal polyposis. INTERVENTION: Saline douching of one side of the nasal cavity, three times per day for 6 weeks. MAIN OUTCOME MEASURES: Presence of adhesions, polyps, crusting, discharge or oedema under endoscopic examination at 3 weeks and 3 months postoperative. RESULTS: Twenty-three patients were recruited. Attendance was 22 patients at 3 weeks and 17 patients at 3 months. At 3 weeks saline douching significantly improved the presence of discharge (P = 0.046) and non-significantly improved the presence of oedema (P = 0.059) with minimal difference with regard to polyps (P = 0.32) and no difference with adhesions or crusting. At 3 months there was minimal difference with regard to crusting (P = 0.18) and oedema (P = 0.32) and no difference with adhesions, discharge and polyps. CONCLUSIONS: Saline douching reduces nasal discharge and may improve oedema during the healing phase following ESS which may represent a possible anti-inflammatory role. No long-term effect was found.

Comparison between two valsalva techniques for improvement of hypopharyngeal nasendoscopy: a preliminary communication.

OBJECTIVE: To compare the nasal valsalva with the trumpet manoeuvre and anterior neck skin traction as aids to nasendoscopic examination of the hypopharynx. DESIGN: Randomised, controlled comparison of examination techniques. SETTING: Single tertiary referral centre. PARTICIPANTS: Twenty-six adult patients requiring hypopharyngeal nasendoscopic examination were recruited. Patients were examined with both techniques in a randomised order that was recorded to video cassette. MAIN OUTCOME MEASURES: Blinded assessment of the percentage visualisation of the pyriform fossae, post-cricoid and upper oesophageal sphincter was carried out by three consultant otolaryngologists independently. RESULTS: Mean percentage scores (and 95% confidence intervals) for nasal valsalva versus trumpet manoeuvre for the three consultants, respectively, were as follows: right pyriform fossa: 77(68, 87) versus 80(71, 91), 61(55, 66) versus 60(54, 66), 46(38, 54) versus 45(37, 54); left pyriform fossa: 76(65, 87) versus 80(69, 91), 59(53, 64) versus 55(49, 61), 42(35, 49) versus 42(35, 50); post-cricoid: 55(44, 67) versus 59(47, 71), 53(46, 60) versus 53(46, 60), 32(25, 39) versus 32(25, 39); upper oesophageal sphincter: 11(1, 21) versus 21(11, 31), 15(9, 21) versus 20(14, 26), 4(0, 8) versus 7(3, 11). No significant difference was found between the two techniques at any subsite. Individual differences were noted in a minority of patients where one or other technique gave a clearly improved view. CONCLUSIONS: The nasal valsalva and the trumpet manoeuvre with anterior neck skin traction are complementary techniques for improving the view of the hypopharynx.

Expression of somatostatin receptors in arginine vasopressin hormone-secreting olfactory neuroblastoma--report of two cases.

OBJECTIVE: Arginine vasopressin hormone-secreting olfactory neuroblastomas are extremely rare, with fewer than twenty cases reported in the literature. Two of these cases, both initially presenting with the syndrome of inappropriate antidiuretic hormone, are presented. The second tumour was successfully identified using somatostatin receptor (octreotide) radiographic scintography. METHOD: The pathological specimens from both cases were examined immunohistochemically for somatostatin receptors. RESULTS: Samples from both cases demonstrated positivity for somatostatin receptors. CONCLUSIONS: This report demonstrates the potential use of somatastatin analogues in the investigation, follow-up and treatment of patients with olfactory neuroblastoma.

Long term results of a randomised prospective study of preservation of the intercostobrachial nerve.

AIM: We have previously reported in a randomised controlled trial comparing intercostobrachial nerve (ICBN) preservation with division that no difference in symptoms was seen between the groups at 3 months follow-up although a reduced area of sensory loss was measured on the arm. To determine if longer follow-up provides evidence for ICBN preservation, follow-up of patients in the trial at 3 years (range 32-38 months) postoperatively was performed. METHODS: Sensory symptoms and deficits, pain, shoulder movements, arm circumference and the presence of neuromas were documented in 73 patients from the original group of 120. RESULTS: No difference in survival or axillary recurrence was observed. The only symptom which differed between the two groups was a subjective assessment of 'different sensation' (P=0.006). No significant difference was observed in other sensory symptoms, pain, shoulder movement, arm circumference or presence of neuromas. A larger area of sensory deficit was measured in women with sacrificed nerves compared to preserved (P=0.009). CONCLUSION: Preservation of the intercostobrachial nerve does not affect patient survival. It improves patient sensory deficit significantly and modestly improves long-term symptoms.

Old age presentation of the Dandy-Walker syndrome associated with unilateral sudden sensorineural deafness and vertigo.

Adult presentation of the Dandy-Walker syndrome is extremely rare. We report a case where the patient was first diagnosed at 75 years of age because of sudden onset of unilateral senorineural deafness and episodic vertigo. He remained well for a further three years until developing the more classical adult presenting features of cognitive impairment, disordered gait and ataxia. The radiological and otological investigative findings are discussed, together with their implications.