Clinical observation of isolated congenital anosmia / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To introduce 8 patients with isolated congenital anosmia and to discuss the clinical manifestations, imaging characteristics and family characteristics of this rarely seen disorder.</p><p><b>METHODS</b>Eight patients with isolated congenital anosmia treated between April 2007 and April 2012 were reviewed retrospectively. There were 4 males and 4 females. A detailed medical history collection, physical examination, nasal endoscopy, T&T and Sniffin'Sticks subjective olfactory function tests, olfactory event-related potentials sinonasal computed tomography scan and sex hormones level monitoring were performed in all patients. Seven cases underwent magnetic resonance image of olfactory pathway examination.</p><p><b>RESULTS</b>All patients were anosmia without evidence of other defects. ENT physical examination, nasal endoscopy and computed tomography scan were normal except 4 cases with obvious nasal septum deviation, 2 cases with concha bullosa. Subjective olfactory test indicated all of them were anosmia. Olfactory event-related potentials were obtained in only 1 patient. Magnetic resonance imaging revealed the smaller or atrophy olfactory bulb and olfactory tract in five cases, the absence of olfactory bulbs and tracts in two case. A female patient did not have MRI examination because of wearing IUDs. Detection of 8 patients of sex hormones were normal. Family characteristics: 3 patients showed family inheritance pattern.</p><p><b>CONCLUSIONS</b>The diagnosis of isolated congenital anosmia should be based on chief complaint, medical history, physical examination, olfactory test, nasal endoscopy, olfactory testing, olfactory imaging and olfactory event-related potentials. Magnetic resonance image of olfactory pathway and olfactory event-related potentials have important value for the diagnosis. More attention should be paid to the genetic susceptibility of the family.</p>

Expression of odorant receptor genes on the olfactory epithelium following olfactory nerve disconnection / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To constitute the animal model of unilateral olfactory nerve transection and observe the expression level and distribution of odorant receptors.</p><p><b>METHODS</b>Thirty-two rats were divided into two groups: the olfactory nerve transection group (20) and the control group (12). The former group received the operation to transect the left olfactory nerve following the left olfactory bulb was exposed under microscope and the latter group did not give any disposal. At every stage of five days, two weeks, four weeks and six weeks after the operation, five rats from the nerve transection group and three from the control group were anaesthetized simultaneously, and olfactory epithelium were taken out after transcardial perfusion, then paraffin imbedding. Coronal sections were sliced for HE staining to observe the thickness changes of the olfactory epithelium, and for in situ hybridization (ISHs) to investigate the expression of olfactory receptor genes (Olr287, Olr226, Olr1493 and Olr1654) in the epithelium, also to evaluate the changes of the expression level and location of the selected receptors during the regeneration of olfactory epithelium.</p><p><b>RESULTS</b>HE staining showed that 5 days after the operation cell quantity and thickness of the olfactory epithelium decreased obviously, which increased gradually 2 or 4 weeks after operation. After 6 weeks' recovery, the thickness of the epithelium could reach the control level. The pattern of cell staining by ISH showed a specific spatial distribution along the anteroposterior (AP) and dorsoventral (DV) axis. Evidence suggested that odorant receptors were distributed in continuous and multiple overlapping bands in the normal or nerve transected-recovered epithelium rather than in the conventionally accepted three or four zones. The data also demonstrated that the distribution of sensory neuron types, as identified and defined by odorant receptor expression, was restored to normal or nearly so by 6 weeks after operation. Likewise, the numbers of probe-labeled neurons in the nerve transected-recovered had an obvious decrease 5 days after olfactory nerve transection. Reactive cells (x(-) +/- s) of Olr1493 in the operated side was (53.9 +/- 19.9), compared with (419.0 +/- 21.2) in the unoperated side, there was statistic significance between them (t = 63.960, P < 0.01). Reactive cells increased gradually according to the regeneration of the epithelium, and were nearly equivalent to the normal side 6 weeks later without significant differentiation (t = 2.600, P > 0.05), according to the absolute positive cells in the operated and unoperated side of (417.8 +/- 32.4) and (445.3 +/- 10.0) respectively.</p><p><b>CONCLUSION</b>The regeneration of the sensory neurons and receptors, both the number and the distribution, can recover to normal after olfactory nerve transection.</p>

Experience in overlay tympanoplasty / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To explore the experience and value of overlay tympanoplasty.</p><p><b>METHODS</b>Sixty-three ears with overlay tympanoplasty were reviewed and followed up for the external auditory canal, tympanic membrane and hearing.</p><p><b>RESULTS</b>The diseases of the patients included middle ear cholesteatoma in 25 ears and chronic suppurative otitis media in 38 ears. The surgical techniques involved three kinds: overlay tympanoplasty, overlay tympanoplasty with canal wall up mastoidectomy and overlay tympanoplasty with canal wall down mastoidectomy. In middle ear cholesteatoma and suppurative otitis media patients, the case received the three techniques are 4, 17, 4 ears and 19, 18, 1 ears respectively. All patients gained stage I incision cure. Followed up for 0.5 to 3.5 years respectively, the external auditory canal was wide and tympanic membrane gained a good shape. The hearing in all case kept intact or increased while hearing decrease did not occur. Complications were free in patients with punctual visit.</p><p><b>CONCLUSIONS</b>Overlay tympanoplasty has positive significance in treating the chronic otitis media with the merits of standard procedure, sufficient operative field and thorough erosion elimination.</p>

Effect of Betamethasone on the cAMP of olfactory receptor neurons in rat / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To observe the effects of Betamethasone on the cyclic adenosine monophosphate (cAMP) in olfactory receptor neurons (ORNs) and explore the possible mechanisms of the recovery of olfactory disorders by steroid treatment</p><p><b>METHODS</b>ORNs membrane was extracted and incubated with 0.1 mg/ml and 1.0 mg/ml Betamethasone. The concentrations of cAMP were measured by enzyme-linked immunosorbent assay at different times.</p><p><b>RESULTS</b>Compared with the control group, all Betamethasone groups showed differences, indicating 0.1 mg/ml (P < 0.05) and 1.0 mg/ml (P < 0.01) Betamethasone could rise the concentration of cAMP. The Betamethasone had obvious effects on cAMP production in rat ORNs and there was a dose-dependent effect. There was no difference between 5 minutes groups and 30 minutes groups.</p><p><b>CONCLUSIONS</b>Steroid hormone could enhance the production of cAMP of ORNs. Steroid hormone may thus contribute to the recovery of olfactory disorders partially, at least, through the effect on AC-cAMP in olfactory transduction.</p>

Clinical features of primary ciliary dyskinesia / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To investigate the clinical features, diagnosis and treatment of primary ciliary dyskinesia (PCD).</p><p><b>METHODS</b>Three cases of PCD received endoscopic sinus surgery and were followed up for life quality and recovery. Among these 3 cases, two were twin brothers and the other girl was twin born with a healthy brother. The mucosa of inferior turbinate was extracted prior to the operation without narcotic and decongestant. The ultrastructure of mucosal cilia was detected with electron microscope. Nine exons of gene DNAH5 and chromosome in one case and her fraternal twin were evaluated.</p><p><b>RESULTS</b>Nasal and sinus CT imaging of the 3 cases showed chronic pansinusitis (1 case accompanied with situs inversus according with the diagnosis of Kartagener syndrome). The nasal polyp was resected, and the sinuses were opened. The twin brothers received the adenoidectomy. All patients felt nasal ventilation improved while the surgical field still covered with thick discharges during follow-up for 2 - 4 years. Ciliary ultrastructures of the three cases showed lateral dynein absent, the sequence of 9 exons of DNAH5 and chromosome presented no change in the fraternal twins.</p><p><b>CONCLUSIONS</b>Surgery could improve the symptoms of sinusitis in PCD. Change of ciliary ultrastructure was an important indication of its pathological changes and molecular biology evaluation needs further study.</p>