Brown–Vialetto–Van Laere syndrome is a rare neurological disorder characterised by pontobulbar palsy and sensorineural hearing loss. Hearing rehabilitation continues to be a challenge because the exact lesion site is unknown.

We examined the clinical and audiological profiles of a case series comprising four siblings with Brown–Vialetto–Van Laere syndrome who had decreased hearing and poor speech discrimination. Audiological investigations revealed normal otoacoustic emissions with absent auditory brainstem responses and middle-ear reflexes in sensorineural hearing loss, suggestive of auditory neuropathy spectrum disorder.

The sensorineural hearing loss in Brown–Vialetto–Van Laere syndrome patients is a retrocochlear pathology resembling auditory neuropathy spectrum disorder, with the lesion being most probably of post-synaptic origin. Early cochlear implantation along with high-dose riboflavin represents a possible rehabilitation therapy. However, further research is needed to confirm this. This report emphasises the need for a thorough neurological evaluation of auditory neuropathy spectrum disorder patients.

Rhabdomyosarcoma is a malignant mesenchymal neoplasm rarely diagnosed in the larynx. Traditionally, it has been treated by radical surgery (i.e. total laryngectomy), followed by radiation and/or chemotherapy. Recent advances suggest that it may be treated with combination therapy comprising high-dose radiation and pulse chemotherapy, with a high success rate.

We report the first documented case of subglottic embryonal rhabdomyosarcoma in an adolescent female treated with chemoradiation alone, and review the literature reflecting a move towards organ preservation.

While surgery has been successfully used to treat this neoplasm, combination therapy, as described in our study, also seems effective and has the added advantage of preserving laryngeal function.

Juvenile nasopharyngeal angiofibroma is a benign, vascular tumour that primarily occurs in adolescent males. Despite its benign nature, aggressive growth patterns can cause potential life-threatening complications. Juvenile nasopharyngeal angiofibroma is normally unilateral, originating from the sphenopalatine artery, but bilateral symptoms can occur if a large tumour extends to the contralateral side of the nasopharynx. This paper presents the first reported case of true bilateral extensive juvenile nasopharyngeal angiofibroma involving clinically challenging pre-surgical planning and surgical strategy.

A 21-year-old male presented with increasing bilateral nasal obstruction and discharge. Examination revealed tumours bilaterally and imaging demonstrated non-contiguous tumours. Pre-operative angiography showed strictly ipsilateral vascular supplies requiring bilateral embolisation. Radical removal performed as one-step, computer-assisted functional endoscopic sinus surgery was performed. The follow-up period was uncomplicated.

This case illustrates the importance of suspecting bilateral juvenile nasopharyngeal angiofibroma in patients presenting with bilateral symptoms. Our management, including successful pre-operative planning, enabled one-step total removal of both tumours and rapid patient recovery.

To highlight the value of sialendoscopy during transoral resection of the sublingual gland for a plunging ranula to prevent iatrogenic injury to the submandibular duct.

The clinical course of a 20-year-old male with a plunging ranula was analysed. The patient underwent transoral resection of the affected sublingual gland and pseudocyst. Sialendoscopy was used to confirm patency of the submandibular duct with placement of a Marchal dilator to aid in preservation of the duct during sublingual gland dissection. The sublingual gland was successfully removed, with no injury to the submandibular duct or lingual nerve. During follow up, the patient had slight numbness to the tip of the tongue, which resolved after 2–3 days. Post-operative examination showed the submandibular duct to be intact and there was no swelling of the submandibular area.

Sialendoscopy-assisted transoral sublingual gland resection for a plunging ranula is a safe and effective technique. Sialendoscopy aids in skeletonisation and preservation of the submandibular duct.

Dermoid cysts in the floor of the mouth are relatively uncommon developmental lesions. They are thought to arise in the midline and along the lines of embryonic fusion of the facial processes containing ectodermal tissue.

A 17-year-old female presented with a 3-month history of a growing, progressive swelling in the mouth floor. Clinical examination revealed a rather large symmetrical, soft swelling in the mouth floor, displacing the tongue superiorly. The fast growing nature and size of the lesion raised suspicion of potential compromise to the airway. Surgical excision was therefore performed.

Differential diagnosis of cystic lesions in the floor of the mouth is of paramount importance, as the recommended surgical techniques vary depending on the anatomical position of the lesions. The intraoral approach is preferred for those lesions that do not extend beyond the mylohyoid muscle boundaries; this leads to a satisfactory cosmetic and functional outcome.