21 Eponyms in ENT

Alexander dysplasia The least severe type of inner ear dysplasia. The cochlear duct and basal turn of the cochlea are usually the only structures affected, resulting in a high-frequency hearing loss.

Alport syndrome Syndrome that consists of haematuria with progressive renal failure (usually starting in the mid-teens), progressive sensorineural hearing loss and retinal flecks. There is a defect in type IV collagen and the symmetrical high frequency sensorineural hearing loss is primarily due to a loss of hair cells. Eighty percent inherited in an X-linked recessive pattern, but there is also autosomal dominant and autosomal recessive inheritance.

Apert syndrome Crouzon’s with syndactyly. A rare autosomal dominant craniofacial disorder.

Arnold’s nerve The auricular branch of the vagus. It arises from the jugular ganglion, passes through the temporal via the mastoid canaliculus and exits the skull through the tympanomastoid fissure. It supplies the skin of the posterior external canal and posterior auricle.

Barre-Lieou syndrome Consisting of sharp pain beginning in the neck and radiating up to the occiput and then forward. It is most common in patients between 40 and 60 years of age. Pain is usually on E one side and aggravated by certain movements of the head. Sensory disturbances, including vertigo, tinnitus and cloudy vision, may accompany the pain. The cause is not known. It is also known as cervical migraine.

Barrett oesophagitis Replacement of the squamous epithelium of the distal oesophagus by columnar epithelium, similar to that which lines the stomach. The most common cause is chronic gastroesophageal reflux, and 2% to 5% of cases may progress to .

Bartholin duct The major duct of the sublingual gland. It is formed by the confluence of several of the more anterior small sublingual ducts (ducts of Rivinus) and empties into either the submandibular duct on directly into the floor of mouth at the plica sublingualis.

Battle sign Ecchymosis over the mastoid process. It is indicative of a temporal bone or posterior fossa fracture.

Behçet syndrome Classically, a symptom complex consisting of oral ulcers, genital ulcers and iritis. The oral lesions can be extensive and may be the initial manifestation of the disease. It is seen most frequently in young adults in Japan and in Mediterranean countries. The cause is unknown; both viral and immune-complex aetiologies have been proposed.

Bezold abscess A subperiosteal abscess of the temporal bone, most commonly found in the region just anterior to the mastoid tip. The cause is usually a mastoiditis with extravasation through the inner bony table into the digastric fossa.

Bing–Siebenmann Complete membranous labyrinthine dysplasia. dysplasia

Boerhaave syndrome Spontaneous rupture of the oesophagus, usually due to severe vomiting.

Bogorad syndrome Profuse lacrimation during eating. It is usually the result of faulty regeneration of autonomic nerves after facial trauma, with parasympathetic fibres originally intended for the salivary glands going to the lacrimal gland instead. It is also known as syndrome of crocodile tears.

Bonnet syndrome A combination of tic douloureux and Horner syndrome.

Bowen disease (i.e. cutaneous SCC in situ). A variant of squamous cell cancer characterized by a full-thickness dysplasia of the epidermis. It is by definition non-invasive, but it can progress to invasive carcinoma. It appears as a red, scaly patch in sun-exposed areas and it can be confused with psoriasis.

Brown sign Blanching of a red or blue mass in the tympanic membrane when air pressure is applied by pneumo-otoscopy. It is indicative of a glomus tympanicum tumour.

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Brown vertical A congenital or acquired pseudo paresis of the inferior oblique muscle, whereby the eye retraction syndrome cannot be elevated beyond mid-gaze. The congenital form is thought to be due to a congenitally shortened superior oblique tendon, whereas the acquired syndrome may be due to either recent trauma (e.g. orbital fracture with entrapment) or previous trauma with formation of adhesions. Broyle’s ligament Anterior commissure ligament of the larynx. Brunner abscess Abscess of the posterior floor of the mouth. Burckhardt dermatitis An eruption of the external ear. It consists of red papules and vesicles that appear after exposure to sunlight. The rash usually resolves spontaneously. Carhart notch Raymond Thomas Carhart in 1950. One of the pioneers of audiology and also first to use the term ‘air-bone gap’. An artefactual loss of bone conduction sensitivity that is greatest at 2 kHz. Most commonly associated with otosclerosis but can occur with otitis media with effusion (OME) and ossicular chain fixation. It is thought to be due to the fact that when bone conduction is tested, a proportion of the sound energy causes the ossicles to vibrate and resonate. The bone E conduction threshold improves on correction of the conductive defect. Churg–Strauss Now known as eosinophilic granulomatosis with polyangiitis (EGPA), is an autoimmune syndrome condition that causes vasculitis of small and medium-sized blood vessels in persons with a history of airway allergic hypersensitivity. It usually manifests in three stages: allergic, eosinophilic and vasculitic. Chvostek sign A facial twitch obtained by tapping the facial nerve in front of the tragus. It can be indicative of hypocalcaemia. Cogan syndrome Typically, a sudden onset of interstitial keratitis and vestibuloauditory symptoms, usually in young persons but sometimes in the elderly. Patients report blurring of vision, orbital pain, vertigo and tinnitus. The symptoms may progress quickly to blindness and deafness, followed by resolution and later relapse. Treatment currently consists of steroids. Cowden syndrome A rare autosomal dominant inherited disorder whose primary ears, nose and throat manifestation are hamartomas of the skin and mucous membranes. Patients with this disease also may have thyroid goitres or adenomas, gastrointestinal polyps and fibrocystic breast disease. They are at increased risk for malignancies in all of these areas, particularly breast cancer. Increased risk of follicular thyroid carcinoma. Crouzon syndrome Named after Octave Crouzon, a French physician. Craniofacial dysostosis affecting the skull and the first branchial arch (precursor of the and ). This autosomal dominant syndrome is caused by a mutation in the fibroblast growth factor receptor. Characterized by hypertelorism, exophthalmos, a hypoplastic mandible and downward-sloping palpebral fissures. Curtius syndrome Hypertrophy of an entire side of the body or of a single part. When it occurs in the face, it is known as congenital hemifacial hypertrophy. Dandy syndrome Oscillopsia caused by bilateral loss of vestibular function, usually as the result of bilateral labyrinthectomy. Darier disease Multiple erythematous, crusted papules distributed over the face and body. These may be (keratosis follicularis) particularly troublesome in the external ear canal. White, ragged papules also may be present in the oral cavity. Dejean syndrome Characterized by exophthalmos, diplopia, superior maxillary pain and numbness along the route of the trigeminal nerve. It is classically caused by a nasal tumour that traverses the pterygopalatine fossa and invades the floor of the orbit. De Quervain’s Fritz de Quervain, 1904, Swiss surgeon from Basel. He also suggested iodised salt for endemic thyroiditis goitre. Subacute granulomatous thyroiditis. Most common cause for painful thyroid. Initial hyperthyroidism followed sometimes by transient hypothyroidism and then return to euthyroid state.

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DiGeorge syndrome Now known as 22q11.2 deletion syndrome. CATCH-22 (= cardiac defects, abnormal facies, thymic hypoplasia, cleft and hypocalcaemia resulting from 22q11.2 deletions). An autosomal dominant defective development of the third and fourth pharyngeal pouches, most frequently manifesting as partial or total agenesis of the thymus (and therefore immune deficiency due to lack of T cells) and parathyroids. Abnormal development of the heart, aortic arch, mandible, external ear and philtrum also may be present. The most common presenting sign is hypocalcaemia in the neonatal period. Elongation of the styloid process or ossification of the stylohyoid ligament, causing recurrent nonspecific throat discomfort, foreign body sensation, dysphagia or facial pain. Surgical shortening of the styloid process can be effective. Epstein pearls Multiple small white nodules on the palate and of newborns. Histologically, they are composed of concentric layers of keratin. No treatment is needed because they disappear spontaneously within a few months. Escherich sign Protrusion of the elicited by percussion of the inner surface of the lips or tongue. It is seen in hypocalcaemia. Fordyce disease A developmental anomaly characterized by enlarged, ectopic sebaceous glands (Fordyce E spots) in the oral mucosa. These glands appear as numerous small yellowish white granules. Frey syndrome Lucia Frey, 1923, Polish neurologist. Died in the Holocaust. Also known as auriculotemporal syndrome. Following parotid surgery, parasympathetic fibres from the auriculotemporal nerve grow into the severed axonal sheaths of sympathetic nerve fibres which innervate the sweat glands. Acetylcholine is the common neurotransmitter. Thus, when a stimulus for salivary flow occurs, sweating of the skin overlying the parotid bed also occurs. Furstenberg test Enlargement of a nasal encephalocele on compression of the internal jugular veins. Garcin syndrome Consists of motor and sensory deficits involving cranial nerves III through XI. Garcin syndrome can be caused by basilar skull fracture with haemorrhage, basal meningitis, cavernous sinus thrombosis or tumours of the parapharyngeal space. It is also known as hemipolyneuropathy. Gardner syndrome A subtype of familial adenomatous polyposis. Characterized first usually by multiple that develop in the skull and facial , including the mandible. It is an autosomal-dominant disease; other symptoms include multiple epidermoid cysts of the skin, papillary thyroid cancer and polyposis of the colon and rectum. There is a tendency for these polyps to become malignant. Goldenhar syndrome Named after Maurice Goldenhar, a Belgian-American ophthalmologist. Also known as oculo-auriculo-vertebral (OAV) syndrome, it is characterized by underdevelopment of the mandible, external ear, orbit, facial muscles and hemivertebrae of the vertebral column. It is a form of hemifacial microsomia. Gorlin syndrome Nevoid basal cell carcinoma syndrome (NBCCS). Autosomal dominant condition that can cause unusual facial appearances and a predisposition for basal cell carcinoma with multiple keratocytic odontogenic tumours. Gradenigo syndrome Count Giuseppe Gradenigo, an Italian otolaryngologist. Otorrhoea, pain in the distribution of the ophthalmic branch of the trigeminal nerve and abducens nerve palsy. Originally caused by an extradural abscess involving the petrous bone, this syndrome is now more commonly caused by a tumour at the petrous apex, such as a cholesteatoma, meningioma or other tumour. It is also known as petrous apex syndrome. Griesinger sign Pain, redness and swelling of the tip of the mastoid, indicative of thrombophlebitis of the sigmoid sinus with involvement of the mastoid emissary veins. Grisel’s syndrome Non-traumatic subluxation of the atlanto-axial joint caused by inflammation of the adjacent tissues. This is a rare disease that usually affects children. The condition often follows soft-tissue inflammation in the neck such as in cases of upper respiratory tract infections, peritonsillar or retropharyngeal abscesses. Post-operative inflammation after certain procedures such as adenoidectomy can also lead to this condition in susceptible individuals such as those with Down’s syndrome.

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Hashimoto’s thyroiditis Described by Hakaru Hashimoto, a Japanese physician, whilst studying in Germany at the University of Gottingen. Returned to Japan at the start of World War I and died of typhoid. Also known as chronic lymphocytic thyroiditis. An autoimmune disease where auto- antibodies to thyroid peroxidase, thyroglobulin and thyroid-stimulating hormone (TSH) are usually present. It is the most common cause of primary hypothyroidism in North America. Hennebert sign In the presence of a normal tympanic membrane, changes in pneumatic pressure produce nystagmus (positive fistula test). The nystagmus is more marked on application of negative pressure. This sign is present with congenital syphilis and is believed to be due to an excessively mobile footplate or to be caused by motion of the saccule mediated by fibrosis between the footplate and the saccule. Hitselberger sign Loss of sensation in the postero-superior part of external auditory meatus. May be caused by a vestibular schwannoma. Hollander syndrome A rare syndrome in which congenital deafness (presumably due to cochlear abnormalities) is linked to the appearance of a goitre in the third decade of life. Thyroid function tests are normal, but biopsy of thyroid tissue shows a partial defect in thyroxine biosynthesis. Horner syndrome Johann Horner, Swiss ophthalmologist. E Characterized by (mild) ptosis, miosis (constricted pupil) and anhidrosis due to damage of cervical sympathetic nerves. Horton neuralgia An autonomic nervous system disorder also known as cluster headache. Patients have unilateral headaches centred behind or close to the eye, along with ipsilateral nasal congestion, suffusion of the eye, and increased lacrimation. Attacks may occur daily for several weeks, then disappear for months or years until another series (cluster) begins. Hutchinson teeth Characterized by small and widely spaced teeth (especially the upper incisors) with notches on their biting surfaces. Only permanent, rather than deciduous, teeth are affected. It is a characteristic sign of congenital syphilis. Jacobson’s nerve The tympanic branch of the glossopharyngeal nerve (cranial nerve IX). It supplies sensory fibres to the mucosa of the middle ear; running with it are preganglionic parasympathetic fibres that leave the middle ear as the lesser superficial petrosal nerve to eventually innervate the parotid. These fibres are cut in an attempt to relieve the gustatory sweating seen in Frey syndrome. Jacod syndrome Consists of progressive ophthalmoplegia, usually starting with paralysis of the oculomotor nerve. This is accompanied or followed by hypesthesia in the distribution of the ophthalmic branch of the trigeminal nerve, exophthalmos and finally involvement of the optic nerve itself. It is caused by a middle cranial fossa tumour that compresses the nerves near the apex of the orbit. It is also called orbital apex syndrome. Jervell and Lange- First described by Anton Jervell and Fred Lange-Nielsen in 1957. Nielsen syndrome A type of long QT syndrome, associated with severe, bilateral sensorineural hearing loss (i.e. (JLNS) always have electrocardiogram (ECG) for paediatric cochlear implants). Autosomal recessive. Kallmann syndrome Consists of hypogonadism secondary to lack of gonadotropins, and anosmia due to agenesis of the olfactory bulbs. It is dominant with variable penetrance. The male-to-female ratio is 3:1. Kartagener’s Manes Kartagener in 1933. syndrome An autosomal recessive primary ciliary dyskinesia with situs inversus. (bronchiectasis, chronic sinusitis and situs inversus). Kiesselbach’s plexus Named after Wilhelm Kiesselbach (1839–1902) a German otolaryngologist. or area A region in the anteroinferior part of the nasal septum where four arteries anastomose to form a vascular plexus of that name. The arteries are: • Anterior ethmoidal artery (from the ophthalmic artery). • Sphenopalatine artery (terminal branch of the maxillary artery). • Greater palatine artery (from the maxillary artery). • Septal branch of the superior labial artery (from the facial artery). It also has been referred to as Little’s area after James L. Little, an American surgeon, who first described the area in 1879.

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Koplik spots Pale round spots on the oral mucosa and conjunctiva that are seen in the beginning stages of measles.

Körner’s septum A remnant of the petrosquamous suture line, which may persist as a plate of bone separating the superficial (squamous) group of mastoid air cells from the deeper (petrous) cells.

Langer lines Tension lines in the skin.

Lemierre’s syndrome André Lemierre, in 1936. French bacteriologist (also known as postanginal septicaemia). Refers to thrombophlebitis of the internal jugular vein. It most often develops as a complica- tion of a bacterial sore throat infection (e.g. Fusobacterium necrophorum) in young, otherwise healthy adults. The thrombophlebitis is a serious condition and may lead to further systemic complications such as bacteraemia or septic emboli.

Lermoyez syndrome Characterized by attacks of tinnitus and deafness followed by a bout of vertigo that surprising- ly relieves the vestibuloacoustic symptoms. It is similar to Ménière's disease. The symptoms in Lermoyez syndrome tend to occur in younger patients than Ménière's disease does, and unlike the latter, gradually resolve over time with no permanent hearing loss.

Lhermitte’s sign Jean Lhermitte, French neurologist in 1924. Sign usually attributed to multiple sclerosis but also a rare complication of radiotherapy to the E head and neck region, causing myelopathy of the spinal cord. Symptoms consist of transient electrical sensation running down the spine and limbs upon neck flexion.

Lillie–Crowe test Used to diagnose unilateral lateral sinus occlusion. Digital compression of the opposite inter- nal jugular vein causes the retinal veins to dilate because the major venous outflow tracts on both sides are now blocked.

Ludwig’s angina German physician, Wilhelm Friedrich von Ludwig who first described this condition in 1836. ankhon = Greek for strangle. A rapidly spreading infection of the submandibular, sublingual and submental spaces. It produces swelling and elevation of the tongue and a brawny induration of the floor of the mouth. It is a diffuse infection, with little or no abscess formation. Its major danger is airway obstruction, and patients may require a tracheostomy until the swelling subsides. The cause of Ludwig angina is usually an odontogenic infection, with streptococci, bacteroides and oral anaerobes being the most common pathogens. Treatment is by antibiotics and drainage of any area of fluctuance.

Maffucci syndrome Characterized by cavernous haemangiomas of the head and neck. Affected patients also have multiple endochondromas, with shortening of the involved bones. Twenty to 40% of patients have malignant degeneration of one or more endochondromas into chondrosarcoma.

Marjolin ulcer A squamous cell carcinoma that arises at the site of an old burn scar, often 20 to 40 years after the initial burn. It is often locally aggressive and metastasizes early.

Meckel’s cartilage The embryonic cartilage from which the mandible, incus and malleus are derived. (First branchial arch.)

Meckel’s cave A diverticulum of dura and arachnoidea encephali mater that lies on the anterolateral surface of the petrous ridge in the middle cranial fossa and contains the trigeminal ganglion.

Melkersson–Rosenthal Characterized by manifestations in childhood or early adolescence as recurring attacks syndrome of unilateral or bilateral facial paralysis with concomitant swelling of the lips and tongue. Affected patients also have a that becomes more prominent with age. It is an autosomal-dominant disease with variable penetrance. The cause is unknown.

Ménière’s syndrome In 1861, Prosper Ménière gave a presentation to the French Academy of Medicine describing a series of patients who experienced episodic vertigo and hearing loss. Ménière’s syndrome is characterised by episodic vertigo, sensorineural hearing loss and tinnitus. A sensation of aural fullness commonly accompanies this classical triad of symptoms.

Michel aplasia Complete labyrinthine aplasia. Middle ear structures may be absent. The external ear and canal are usually normal in appearance.

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Mikulicz cells Named after Johann von Mikulicz, a Polish professor of surgery. Large vacuolated histiocytes containing phagocytised bacteria, characteristic of rhinoscleroma.

Mikulicz disease Bilateral, recurrent inflammatory enlargement of the lacrimal and salivary glands. When due to a manifestation of some other systemic disease, such as lymphocytosis or tuberculosis, it is known as Mikulicz syndrome. Pathology shows a diffuse lymphocytic infiltrate.

Möbius syndrome Congenital facial paralysis (usually bilateral) with paralysis of the abducens nerve and sometimes other oculomotor nerves. Pathologically the few cases studied have usually shown hypoplasia of the involved brainstem nuclei.

Mondini dysplasia A cochlea with incomplete partitioning and a reduced number of turns, an enlarged vestibular aqueduct and a dilated vestibule. Sensorineural hearing loss may present in childhood and can be associated with minor head trauma. There is also a predisposition to perilymph fistulae and meningitis.

Sinus of Morgagni A defect between the upper edge of the superior constrictor muscle and the buccopharyngeal fascia through which the eustachian tube passes.

E Nager syndrome Characterized by facies similar to those seen with Treacher Collins syndrome. Affected patients also present with preaxial upper limb defects, microtia, atresia of the external auditory canals and malformation of the ossicles. Conductive and mixed hearing losses may occur. The inheritance pattern has not been determined, because most cases are sporadic. Also known as acrofacial dysostosis.

Oliver sign A pulling sensation felt in the larynx and trachea due to an aortic arch aneurysm. It is most evident when the head is extended.

Ondine’s curse Named after the myth of Ondine, a water nymph who had an unfaithful mortal lover. He swore to her that his ‘every waking breath would be a testimony of [his] love’, and upon witnessing his adultery, she cursed that if he should fall asleep, he would forget to breathe. Eventually, he fell asleep from sheer exhaustion, and his breathing stopped. Failure of respiratory drive, especially during sleep. Congenital central hypoventilation syndrome (CCHS). Treatment is tracheostomy with mechanical ventilation at night, although bilateral phrenic pacing has been used successfully in some patients.

Orphan Annie-eye Large nuclei which appear empty. Typically seen in papillary thyroid carcinoma but also seen nuclei in autoimmune thyroid disease and polymorphous low-grade adenocarcinoma. Named after a comic strip character popular in the 1930s, Little Orphan Annie, who was depicted with vacant circles for eyes.

Ortner syndrome A rare cause of hoarseness in infants with congenital cardiac disease. Compression of the left recurrent laryngeal nerve between the aorta and a dilated pulmonary artery results in paralysis of the left vocal cord.

Osler–Weber–Rendu Hereditary haemorrhagic telangiectasia, characterized by punctate telangiectasias disease usually developing around puberty and commonly seen in the oral and nasal mucosa and tongue. Patients commonly present with epistaxis and may have visceral arteriovenous malformations (AVMs). Autosomal dominant inheritance.

Pancoast tumour Henry Pancoast, American radiologist. Pulmonary sulcus tumour of pulmonary apex. Usually non-small cell carcinoma.

Pancoast syndrome Shoulder pain radiating down the distribution of the ulnar nerve of the arm, caused by local extension of a tumour in the apex of the lung that eventually invades the brachial plexus. Horner syndrome, indicating involvement of the cervical sympathetic chain, also may be present.

Passavant ridge A horizontal mucosal fold across the posterior pharynx that is the point of contact by the soft palate when the nasopharynx is closed during the act of swallowing. Whether this is an active or passive fold is still unresolved.

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Pendred syndrome An autosomal-recessive syndrome consisting of a bilateral congenital sensorineural hearing loss, enlarged vestibular aqueducts and the appearance of a goitre years later in mid-childhood. T4 levels are usually low to absent, and a perchlorate test is diagnostic. The hearing loss is non-reversible. Peutz–Jeghers An autosomal-dominant disorder whose two main components are benign polyps of the syndrome intestinal tract and mucocutaneous melanotic macules. The latter bring these patients to the attention of the otolaryngologist and may appear at any time from infancy to adulthood. The macules are most common around the facial orifices (perioral, perinasal and periorbital) and on the buccal mucosa. This syndrome is not to be confused with Gardner syndrome, in which the intestinal polyps tend to become malignant. Pfeiffer syndrome Named after Rudolf Arthur Pfeiffer. An autosomal dominant craniosynostosis with abnormalities of the hands and feet. Associated with mutations in fibroblast growth factor receptor. Pierre Robin sequence Named after a French dental surgeon. Consists of micrognathia (relative) and cleft palate. There is no sex predilection. The cause is believed to be arrested intrauterine development; the syndrome may occur as an isolated triad or as part of a larger constellation of defects. Affected infants often present with choking and aspiration, presumably due to the hypoplastic mandible and glossoptosis. E Plummer–Vinson The disease is named after two Americans: the physician Henry Stanley Plummer and the syndrome surgeon Porter Paisley Vinson. It is occasionally known as Paterson–Kelly or Paterson–Brown– Kelly syndrome in the United Kingdom, after Derek Brown–Kelly and Donald Ross Paterson. Characterized by pale skin, dysphagia, atrophy of the tongue papillae, and sometimes oral or angular , found almost exclusively in middle-aged women. This condition is primarily due to an iron-deficiency anaemia, with a deficiency of vitamins and protein apparently also playing a role. The dysphagia is attributed to the formation of an oesophageal web, although how or why the web is formed is unknown. There also may be an increased risk of postcricoid cancer in these patients. Treatment is iron supplementation. It is also known as sideroblastic dysphagia. Prussak’s space The upper tympanic recess, bounded by the pars flaccida laterally, the neck of the malleus medially, the lateral mallear ligament superiorly, and the lateral process of the malleus inferiorly. Because it has only small openings anteriorly and posteriorly, it can be a common site for cholesteatoma. Raeder syndrome Characterized by severe unilateral orbital pain with accompanying miosis and ptosis, but not anhidrosis. Paralysis of one or more ocular nerves may be seen. It is also known as incomplete Horner syndrome. Ramsay Hunt Herpes zoster oticus. Classically described as a unilateral otalgia and facial paralysis syndrome accompanied by a vesicular rash in the external ear. It is caused by a herpetic infection of the cranial nerves. Patients also can have a sensorineural hearing loss, vertigo and tinnitus. There have been some reports of improvement in symptoms following treatment with oral acyclovir. Rathke’s cleft cyst A benign growth found on the pituitary gland in the brain, specifically a fluid-filled cyst in the posterior portion of the anterior pituitary gland. It occurs when the Rathke’s pouch does not develop properly, and ranges in size from 2 to 40 mm in diameter. Reed–Sternberg cells Binucleate or multinucleate B cells with prominent nuclei characteristic of Hodgkin’s lymphoma. Riedel’s thyroiditis Bernhard Riedel, a German surgeon, first recognized the disease in 1883. An extremely rare form of chronic thyroiditis in which a fibrotic reaction of unknown aetiology replaces most thyroid tissue and frequently extends out of the thyroid capsule to compress adjacent structures. Patients are usually middle-aged women and may present with a painless neck mass, dysphagia, or hoarseness. Now thought to be a type of immunoglobulin G4 (IgG4) disease. Ducts of Rivinus Small salivary ducts that pass directly from the upper border of the sublingual gland to empty into the oral cavity. Also see Bartholin duct.

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Node of Rouvier Lateral retropharyngeal node. It is a common target of metastases in nasopharyngeal carcinoma. Fissures of Santorini Fissures in the anterior bony external auditory canal leading to the parotid region. Scarpa’s ganglion Antonio Scarpa, Italian anatomist and surgeon. The ganglion containing the cell bodies of the bipolar cells making up the vestibular nerve. It is located at the lateral end of the internal auditory canal. Scheibe aplasia Also known as cochleosaccular dysplasia, it is the most common type of cochlear dysplasia. The bony labyrinth is completely formed, as are the utricle and semicircular canals. The pars inferior (saccule and cochlear duct) are undifferentiated, and the membranous cochlea is malformed. Sjögren syndrome All known as sicca syndrome and described in 1933 by Henrik Sjögren, a Swedish ophthalmologist. Defined by the presence of two or more of the following symptoms: dry eyes (keratoconjunctivitis sicca), dry mouth (xerostomia), painless swelling of the parotid glands and polyarthritis. Most frequently seen in middle-aged women, its aetiology is unknown, although an autoimmune cause is suspected. E Sluder neuralgia Similar to Horton neuralgia (cluster headache) and also known as sphenopalatine neuralgia. It is sometimes treated by vidian neurectomy. Stickler syndrome It was first studied and characterized by Gunnar B. Stickler in 1965. Hereditary progressive arthro-ophthalmopathy. A group of hereditary conditions affecting collagen. Stickler syndrome is characterized by distinctive facial abnormalities, ocular problems, hearing loss, and joint problems. Some subtypes have an autosomal dominant inheritance pattern while others are autosomal recessive. Sturge–Weber Characterized by a unilateral port-wine stain somewhere within the distribution of the syndrome trigeminal nerve. It is apparently a defect of the mesodermal component of blood vessels and is further characterized by angioma of the leptomeninges, orbit, mouth and nasal mucosa. Intracerebral calcifications seen on plain radiographs are diagnostic. Symptoms include seizures, hemiparesis and glaucoma. There is no known treatment. Tapia’s syndrome Spanish otolaryngologist Antonio Garcia Tapia in 1904. Unilateral extracranial nerves X and XII palsy. It was first described in two bullfighters who were gored in the neck. The syndrome also may be caused by a tumour in the deep lobe of the parotid and has been reported in brachial plexus blocks and post-intubation. Tolosa–Hunt Unilateral retroorbital pain and ophthalmoplegia, which may be either steadily progressive syndrome or recurrent. It is thought to be due to inflammation of the cavernous sinus by any of several causes. Thornwaldt cyst A cyst that arises from the pharyngeal bursa (pouch of Luschka). Its location is in the midline of the posterior nasopharynx, surrounded by adenoid tissue. It can become infected and present as a nasopharyngeal mass. Treacher Collins Also known as mandibulofacial dysostosis, an autosomal dominant disorder characterized syndrome in children by a severely hypoplastic mandible. Infants sometimes require tracheostomy because of lack of anterior support of the tongue. Other features of the syndrome are down-sloping palpebral fissures, defects of the external ear, auditory canal, and ossicles, and occasionally, cleft palate. TCOF1 mutations are the most common cause of the disorder. Trotter syndrome May be seen with tumours of the nasopharynx that block the eustachian tube and produce (sinus of Morgagni a conductive hearing loss secondary to middle-ear fluid. Other symptoms may be pain in the syndrome) distribution of the ophthalmic branch of the trigeminal nerve, decreased mobility of the soft palate, and possibly . Trousseau’s sign Armand Trousseau, a French physician in 1861 described tetany caused by a tourniquet placed around the arm of a patient with hypocalcaemia.

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Tullio phenomenon First described in 1929 by the Italian biologist Prof. Pietro Tullio (1881–1941). During his experiments on pigeons, Tullio discovered that by drilling tiny holes in the semicircular canals of his subjects, he could subsequently cause them balance problems when exposed to sound. Said to be present when a loud noise precipitates vertigo. It can be present with congenital syphilis or a perilymph fistula. The tympanic membrane and ossicular chain must be intact with a mobile footplate.

Turner syndrome Gonadal dysgenesis syndrome. The phenotype is always female, and the karyotype is usually XO. Signs and symptoms include growth retardation, webbed neck, primary amenorrhea, lack of secondary sex characteristics, cardiac abnormalities and ocular problems. Treatment is oestrogen replacement.

Usher syndrome Described by Charles Usher, a Scottish ophthalmologist in 1914. A leading cause of deaf-blindness. Characterized by a multitude of progressive sensory deficits, including sensorineural hearing loss, retinitis pigmentosa and anosmia. The mechanism is postulated to be degeneration of both central pathways and neuroectodermal tissue at the sensory end-organ. Autosomal recessive.

Vail syndrome A unilateral, nocturnal pain in the nose, face and eye along with rhinorrhea and symptoms of sinusitis. It is postulated to be due to irritation of the vidian nerve secondary to sphenoid E sinusitis. It is also known as vidian neuralgia.

Vernet syndrome Another jugular foramen syndrome with involvement of cranial nerves IX, X and XI. It may be due to trauma, an aneurysm, a tumour, or other conditions.

Vidian nerve Also called the nerve of the pterygoid canal. It is formed by the union of the greater and deep petrosal nerves. It is sometimes sectioned in an attempt to control severe vasomotor rhinitis.

Villaret syndrome Similar to Vernet syndrome, with the addition of the deficits associated with Horner syndrome, indicating involvement of the cervical sympathetic chain. This syndrome suggests a lesion distal to the jugular foramen, usually in the retrostyloid area.

Waardenburg Described by Dutch ophthalmologist Petrus Johannes Waardenburg in 1951. syndrome An autosomal dominant group of disorders characterised by sensorineural hearing loss, which may be present at birth or develop later in life, and changes in pigmentation. Other manifestations of the syndrome include hypertelorism (type I Waardenburg), heterochromia iridis and partial albinism which often expressed as a white forelock. Hirshsprung’s disease is associated with type IV.

Wallenberg syndrome Also known as lateral medullary syndrome or posterior inferior cerebellar artery syndrome. Characterized by vertigo, nystagmus, nausea and vomiting, Horner syndrome, dysphagia, dysphonia, falling to the side of the lesion, and loss of pain and temperature sense on the ipsilateral face and contralateral side below the neck. It is caused by thrombosis of the posteroinferior cerebellar artery, leading to ischemia of the ipsilateral brainstem.

Weber syndrome Classic symptoms of damage to the oculomotor nerve at its emergence from the mid-brain, combined with damage to the adjacent pyramidal tract prior to its decussation. In addition, there is ipsilateral paralysis of cranial nerve III and contralateral paralysis of the extremities, face and tongue.

Wildervack (cervico- A congenital syndrome seen primarily in females, presenting at birth with a mixed hearing oculo-acoustic) loss, a short, webbed neck with fused cervical vertebrae, and bilateral abducens palsy. It is syndrome similar to Klippel Feil syndrome.

Winkler disease Chondrodermatitis nodularis chronica helicis. A disease of the helix of the ear, seen primarily in men over 40 years of age. It presents as one or more painful nodules. Pathologically, both the skin and the perichondrium are involved. Treatment is surgical excision, with recurrences common.

83 Roland et al., Key Topics in Otolaryngology, 3rd Ed. (ISBN 978-3-13-240477-9), copyright © 2019 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.